A 59-year-old female with a history of Crohn’s disease and hepatic cirrhosis
presented to the dermatology clinic for sudden-onset, generalized warts. Physical
exam was remarkable for numerous tan-colored, verrucous papules on the head, trunk,
and extremities ().
Two months prior, she was started on ustekinumab for her Crohn’s disease which had
been quiescent for 3 years without immunosuppressive therapy post-ileocolectomy. The
administration of ustekinumab involved a 390-mg intravenous infusion for induction
therapy, followed by a 90-mg subcutaneous injection every 8 weeks. The patient’s
other medications included gabapentin, trazodone, rifaximin, zinc sulfate,
lactulose, spironolactone, and torsemide, all of which she had used for years and
tolerated well. She also had not started any new medications while receiving
treatment with ustekinumab.
Biopsies of the verrucous lesions were performed. Histopathologic analysis was
consistent with HPV infection () and polymerase chain reaction confirmed the presence of
several HPV types including HPV types 2, 3, 16, 66, 93, 98, and 150. Among the 10
biopsied samples, HPV type 2 was the most frequently detected type with six positive
samples (). Due
to suspicion for ustekinumab-induced disseminated verrucosis, the medication was
stopped. Cessation of the ustekinumab, in addition to treatment with liquid nitrogen
cryotherapy and aminolevulinic acid photodynamic therapy, initially resulted in a
significant decrease in the number of verrucae. However, the patient experienced a
flare of her Crohn’s disease and was subsequently restarted on ustekinumab by her
gastroenterologist. This was associated with the development of numerous additional
verrucae on the face, neck, trunk, and extremities. Despite new and ongoing
verrucae, the patient decided to remain on ustekinumab. She continues to receive
treatment with cryotherapy and aminolevulinic acid photodynamic therapy.


A 49-year-old premenopausal woman was referred to the endocrinology outpatient department with severe low backache and a 10 cm height loss over the preceding 10 years (from 160 to 150 cm). Evaluation at her hometown revealed severe osteoporosis, for which she had received three doses of parenteral zoledronate annually, followed by oral alendronate 70 mg once weekly for 7 years. There was no history suggestive of secondary osteoporosis or long bone fractures. There was no history of similar disease in first-degree relatives.
On examination, she was obese with a body mass index (BMI) of 31.6 kg/m2 and height of 150 cm []. Systemic examination was unremarkable except for kyphoscoliosis. On biochemical evaluation of blood bone mineral parameters, she was found to have an albumin corrected calcium of 8.9 (N: 8.3–10.4) mg/dL, fasting phosphorus of 3.1 (2.5–4.6) mg/dL, 25 OH Vitamin D level of 29 (>30) ng/mL, and parathormone (PTH) of 26 (8–50) pg/mL. Bone turnover markers were suppressed at the time of presentation (alkaline phosphatase [ALP] 49 (40–125) U/L, P1NP 17.7 [15.1–58.3] ng/ml, CTX 103 [137–573] pg/mL) []. X-ray of the thoracic and lumbar spine showed multiple vertebral compression fractures []. Genetic screening for osteogenesis imperfecta was negative. Dual-energy X-ray absorptiometry scan (DXA) showed osteopenia at the neck of the femur and lumbar spine. The BMD at the neck of the femur and lumbar spine were 0.684 and 0.825 g/cm2, respectively. In view of suppressed bone turnover markers and ongoing height loss, she was initiated on subcutaneous teriparatide at a dose of 20 µg daily in addition to 1000 mg of elemental calcium and cholecalciferol 1000 IU/day. However, despite 1 year of treatment, there was further height loss of 2 cm (150 to 148 cm). Even though lumbar spine BMD increased by 4.24%, there was a BMD loss of 7.3% at the neck of the femur.
Due to ongoing height loss and elevated levels of bone turnover markers (ALP 125 [40–125] U/L, P1NP 457 [15.1–58.3] ng/ml, and CTX 1381 [137–573] pg/mL) at the end of 1 year of anabolic therapy, denosumab (receptor activator of nuclear factor κB ligand [RANKL] inhibitor antiresorptive agent) subcutaneously at a dose of 60 mg at 6 monthly intervals was added. Subsequent to the first dose of denosumab added to her ongoing therapy with teriparatide, she had marked improvement in symptoms and no further height loss was recorded thereafter.
Three months after initiation of denosumab therapy continued along with teriparatide, alkaline phosphatase, P1NP, and beta crosslaps levels were 83 U/L, 110 ng/mL, and 451 pg/mL, respectively. BMD at the neck of femur and L1–L4 were 0.680 and 0.934 g/cm2, respectively, after 6 months of initiation of denosumab []. Bone formation markers were not improved with the combination therapy; however, there was improvement in BMD. Combination therapy resulted in marked symptomatic improvement, with no further height loss. The plan is to continue the combination therapy (teriparatide and denosumab) for a total duration of 2 years and thereafter to give denosumab or bisphosphonates.


A 31-year-old Arab man, with no past medical history or family history of Behcet's disease, presented to our emergency department with a 4-day history of a diffuse cervico-facial edema associated with odynophagia without fever, dyspnea, or dysphonia.
The clinical examination revealed a diffuse cervical edema extended from the submandibular region to the supraclavicular and suprasternal regions. The edema was bilateral, symmetrical, soft, poorly limited, and noninflammatory. It was a nonpitting edema (Figure ). There was no cervical lymphadenopathy. The chest examination showed a collateral venous circulation. The cardiopulmonary auscultation was normal.
Oropharyngeal and oral cavity examination showed multiple painful canker sores on the tongue (Figure ) with a regular, symmetrical, and noncompressive bulging of the posterior wall of the oropharynx. There was no limitation of the mouth opening or tonsil bulging. The rest of the examination showed no signs of local infection.
The laboratory workup revealed a biological inflammatory syndrome (C-reactive protein 70 mg / l and sedimentation rate 40). Complete blood count was normal.
The cervico-thoracic computed tomography (CT) scan with contrast showed a retropharyngeal thickening extended to the parapharyngeal space with thrombophlebitis of the superior vena cava (Figure ). Cervical magnetic resonance imaging (MRI) showed a retropharyngeal hyperintense lesion on diffusion weighted-sequence: the radiologic findings were consistent with a diagnosis of a retropharyngeal edema (Figure ). Laboratory thrombophilia testing was normal.
Upon further questioning, we found that the patient had recurrent oral and genital aphthosis lasting for 6 months. Ophthalmologic examination was normal (didn't reveal uveitis). Skin examination was unremarkable. The diagnosis of Behçet's disease was evoked, then confirmed after grouping a set of clinical and biological criteria (oral and genital ulcers, thrombophlebitis, and positive pathergy test).
The patient was treated with prednisolone and colchicine, as well as anticoagulation (vitamin K antagonist). Clinical evolution was favorable after 3 months of follow-up.


The authors report a case of a 73-year-old Caucasian male with background history of type 2 diabetes and arterial hypertension diagnosed with prostatic adenocarcinoma in December 2018. Radical prostatectomy was performed, and the patient was started on hormone therapy with cyproterone and adjuvant radiotherapy (March 2019). He had 1-month history of night sweats and headache and 1-week history of moderate, nonirradiating pain in the anterior thighs and low-grade fever. At examination, the patient was pale, sweaty and had a grade 2/5 panfocal heart murmur. Laboratory tests revealed normocytic normochromic anemia, erythrocyte sedimentation rate (ESR), transaminases, C-reactive protein (CRP), and procalcitonin. Cultures were drowned, and empirical antibiotics were started. The head CT scan and lumbar CT scan were unremarkable. Blood cultures and serologic tests for atypical agents were negative. A transthoracic echocardiogram was performed and showed no valvular vegetations. A thoracic-abdominal-pelvic CT scan was performed and showed only moderate hepatomegaly. After ten days of empirical antibiotic with doxycycline and gentamicin, the patient maintained low-grade fever and pain in the anterior thighs with worsening of anemia, SR, and transaminases. The CRP and procalcitonin remained unchanged, and the multidisciplinary team decided to suspend local radiotherapy.
A bone scintigram was performed and suggested the hypothesis of aseptic femoral head necrosis that was excluded with hip MRI. With development of mandibular claudication during the hospital stay, a Doppler ultrasound of the temporal arteries was performed and revealed periluminal hypoechogenic halo reflecting arterial wall edema more pronounced in the left temporal artery (Figure ).
The patient was started on 1 mg/kg of prednisolone with clinical improvement at the first 48 hours and resolution of laboratory abnormalities. It was not possible to perform the temporal artery biopsy until the 10th day of therapy, and it showed only slight lymphocytic infiltration.
Local radiotherapy was restarted, and the patient was discharged home.
Three months later, the patient restarted bilateral hip pain and a lumbosacral spine MRI was performed and revealed new osteoblastic lesions in S1, S2, and S3 compatible with bone metastasis.


We present a 52-year-old right-handed Caucasian male with a medical history of HIV, hepatitis C, hypertension, and type II diabetes with a chief complaint of sudden onset severe upper back pain. The patient stated that the upper back pain started while he was conducting a Zoom lecture as a math teacher. He denied any trauma or injuries. He also denied any regular use of anticoagulant medications, but did take aspirin 81 mg daily for general cardiovascular health. He also denied any subjective fever or chills. He stated that the pain was 8/10 in severity. Within hours, he started to notice weakness in his right and right hand which prompted him to call the ambulance and was brought to the hospital. As the weakness became more severe, he began to develop numbness in the right hand and right leg. The patient was admitted and a magnetic resonance imaging (MRI) of the cervical spine was performed.
Radiographically, MRI of the cervical spine without contrast revealed an epidural fluid collection identified posterior to the cord at the C4-C7 levels measuring up to 1 cm thick []. The cord appeared compressed and was most pronounced at the C6 and C7 levels with mild increased cord signal. The signal characteristics were compatible with epidural hematoma.
The patient was emergently taken to the operating room for posterior C5-C7 decompressive laminectomy and evacuation of intra-SEH. On removal of the lamina and ligamentum flavum, there was visualization of epidural hematoma. The purple, clotted, thick, gelatinous material was completely evacuated and sent to pathology for confirmation. There was no obvious vascular malformation noted and there did not appear to be any trauma in the adjacent soft tissues. There did not appear to be any unusual bleeding problems intraoperatively. Complete evacuation of hematoma and decompression was achieved. The histopathological specimen consisted of a 2 × 2 × 1 cm aggregate of dark brown-purple clotted blood fragments. The pathological diagnosis confirmed SHE with no malignancy cells. The patient was returned to his hospital room for continued recovery and physical therapy rehabilitation. He was subsequently discharge to acute rehabilitation facility. The above patient represents a case of idiopathic SSEH.


We present a 72-year-old right hand dominant Caucasian female transferred from outside hospital status – post sudden onset severe left neck pain and left arm pain. She stated that her neck pain started the night prior and suddenly started radiating into her left shoulder and arm which was associated with progressive weakness. Her pain was made worse by any movement. She denied any preceding trauma. She denies any medical history other than hypertension. Of note, she stated that she does take 325 mg aspirin daily for joint pain. She denies having any known bleeding disorder but stated that she tends to bruise easily. Her coagulation laboratories indicated aspirin therapeutic response causing platelet dysfunction: platelet EPI >300 (normal 72–184) and platelet function aspirin 560 (normal 570–675). Her platelet count was 400 and other laboratory work noncontributory. Her physical examination was significant for having motor deficits as follows: left upper extremity: 4/5 deltoid, 4/5 bicep, 3/5 triceps, 2/5 wrist extension, 2/5 wrist flexion, 4/5 handgrip, and 2/5 interosseous muscles, left lower extremity: 4+/5 hip flexion, 5/5 knee flexion, 5/5 knee extension, 4+/5 dorsiflexion, 5/5 plantar flexion, and 5/5 extensor hallucis longus. She also had sensory deficits in the left upper extremity which displayed decreased sensation to pinprick in a nondermatomal distribution.
Radiographically, MRI of the cervical spine showed an epidural hematoma dorsal to the cord at the C3-T2 levels measuring up to 6 mm thick []. It lied eccentric toward the left with mild-to-moderate compression on the cord. There was no definite cord edema, abnormal enhancement, or major ligamentous injury. MRA of the neck was negative for any abnormal vascularity.
She was taken to surgery within hours of presentation for emergent posterior C3-C7 decompressive laminectomies and evacuation of intra-SEH. There were findings of dorsal purple clotted epidural hematoma that was eccentric to the left. Operatively, there was complete decompression achieved and hematoma was entirely evacuated. Baseline intraoperative neuromonitoring showed decreased motor signals in bilateral upper extremities, left worse than right. The SSEP sensory signals were normal and stable throughout the surgery. After decompression and evacuation, there was identifiable improvement in bilateral upper extremity motor signals on intraoperative neuromonitoring.
Postoperatively, within the first 24 h period, she had near resolution of her weakness with only residual weakness of 4+/5 wrist extension, 4+/5 wrist flexion, 4/5 handgrip, and 4/5 interosseous muscle. She continued to have left upper extremity decreased sensation to pinprick testing in nondermatomal fashion that was stable. She worked with the physical and occupational therapy teams and was subsequently discharged home with outpatient physical therapy and recommendations to discontinue her daily aspirin use. The above case represents SSEH likely resultant from daily aspirin usage.


A 50-year-old male, father of a β-thalassemia major patient, reported having HbF, i.e., 94.90% without any symptoms while being investigated for the thalassemic patient's family status.
Wife is a known case of thalassemia minor with the history of three miscarriages. Family has an 8-month live baby with thalassemia major. No other live member (maternal/paternal) was available for the further investigation.
Father's hemoglobin by high performance liquid chromatography (HPLC)/electrophoresis is HbF - 94.90%, Hb A - 2.60%, HbA2-2.0%, and others - 0.50%. Hb concentration - 15.10 g% with erythrocytosis (RBC count - 6.37 million/mm3). Child – hemoglobin HPLC/electrophoresis – HbF - 91.60%, Hb A - 6.0%, HbA
2-2.1%, and others - 0.30%. Mother – reported as β-thalassemia minor.


A 42-year-old man presented with increasing dyspnoea, wheezing and haemoptysis. He had presented with right testicular carcinoma 2 years before, which was treated with surgical resection and adjuvant chemotherapy. Computed tomography showed a mass in the right main bronchus, and possibility of metastasis was considered.
Urgent rigid bronchoscopy revealed a globular tumour blocking 90% of the right main bronchus (). The tumour was completely resected with the diathermy snare and the tumour bed was treated with diathermy.
Histological examination showed spindle shaped cells with prominent nucleoli and eosinophilic cytoplasm arranged in short fascicles with occasional mitoses in the tumour cells ( and b). The stroma was infiltrated with lymphocytes, histiocytes and occasional eosinophilic granulocytes. On immunohistochemistry (IHC), tumour cells stained positive for ALK-p80 (). ALK fluorescence in situ hybridisation (FISH) was positive with split signals. FISH revealed a rearrangement in ALK (), and hybrid capture based next generation sequencing (NEOselect) showed EML4-ALK1 fusion. Diagnosis of inflammatory myofibroblastic tumour was made.
Bronchoscopy after 1 month showed necrotic tissue at the tumour base. Follow-up with bronchoscopy and computed tomography has revealed no recurrence in 38 months ().


A 49-year-old man presented with haemoptysis and breathlessness of 1-month duration. Computed tomography of the chest showed a mass in the middle lobe bronchus with atelectasis of the middle lobe.
Urgent bronchoscopic intervention was planned due to increasing dyspnoea. A flexible bronchoscope was passed through the channel of the rigid bronchoscope. A smooth rounded tumour covering the right middle and lower lobe bronchi was seen (). The tumour was removed with the diathermy snare and the tumour base at middle lobe carina (RC2) was treated with diathermy. Bleeding was controlled with endobronchial instillation of epinephrine, tranexamic acid and use of a bronchial blocker.
Histology showed epitheloid cells with granular cytoplasm arranged in short fascicles with infiltration of lymphocytes. Tumour cells were negative for S100 on IHC, ruling out a granular cell tumour. Subsequent ALK-p80 was positive. FISH- and hybrid capture-based next generation sequencing confirmed the diagnosis of EML4-ALK1 fusion-positive IMT.
Rigid bronchoscopy was repeated after a month. Abnormal mucosa on the roof of right middle lobe bronchus and RC2 was treated with diathermy and cryotherapy. Biopsies showed no residue. Bronchoscopy at 5 months showed an oedematous right middle lobe opening, but biopsies were clear of residual tumour. Follow-up with computed tomography scan and bronchoscopy showed no recurrence in 40 months ().


A 16-year-old girl presented with cough and was diagnosed as having pneumonia that was treated with antibiotics. 1 month later she presented with pneumomediastinum. Computed tomography scan showed a tumour in the distal left main bronchus with atelectasis of the left lower lobe.
Rigid bronchoscopy revealed a tumor in the distal left main bronchus, vascular and bleeding easily on touch. The surface of the tumor was coagulated with cautery and mechanical debulking with the rigid scope was done ( and f). Bleeding from the tumour bed at the ventro-medial aspect of distal left main bronchus was controlled with diathermy and pressure with a bronchial blocker. The procedure was stopped at this point and the patient was advised a follow-up.
Histology showed spindle shaped tumour cells with elongated nuclei, prominent nucleoli and eosinophilic cytoplasm arranged in a herring bone pattern, with infiltrates containing lymphocytes and occasional eosinophilic granulocytes. The cells stained positive forALK-P80 on IHC, FISH- and hybrid capture-based next generation sequencing showed an EML4-ALK1 fusion positive IMT.
After 2 months, repeat bronchoscopic treatment for residual tumour with application of diathermy was done. Biopsies after 4 months were clear of tumour. After 7 months, a recurrence was noted at the distal left main bronchus that was treated with cryotherapy and diathermy. Biopsies at 9 and 12 months were clear of tumour. After 18 months, computed tomography showed recurrence in the distal left main bronchus and bronchoscopic biopsies confirmed ALK positive IMT. The patient was referred for sleeve resection of the distal left main bronchus and surgical resection was achieved with margins being clear of tumour. In the resected tumour, the EML4-ALK1 fusion was confirmed. FISH for ROS1 and RET mutations were negative. The patient was asymptomatic and computed tomography scan 30 months after surgery has shown no evidence of recurrence.


A 40-year-old Mexican woman presented to our hospital with a giant tumor in her right breast. The patient had noticed the mass 3 years earlier; 1 year later, blisters had appeared on the nipple. In the last year, the mass had presented with erythema, a venous network, a foul smell, and skin ulceration with bleeding, and it had also increased considerably in size. Due to the rapid growth, the patient consulted us for help. The family and personal history did not provide any information relevant to the case. Physical examination revealed an enlarged right breast, approximately 25 × 22 cm, with irregular margins, erythematous and hyperemic skin, ulceration of the nipple with hemorrhagic discharge, pain on palpation, and the axillary region without nodes (Fig. ). Laboratory tests only showed leukocytosis (12,700/μL) with neutrophilia (9,700/μL), without any other significant finding. Sonography of the breast showed a huge mass with multiple areas of cystic degeneration alternating with solid tissue, poorly defined margins, irregular vascularity, and thick hyperechogenic septa. An ultrasound-guided biopsy was performed, and the histopathology results showed a fibroepithelial neoplasm with hyalinized, myxoid, and hypercellular stromal areas, compatible with fibroadenoma, with a Ki-67 proliferation index of 2% (Fig. , ).
Subsequently, 18F-FDG PET/CT showed a right breast mass that measured 24.6 × 17.3 cm in the axial plane and 22.5 cm in the cephalocaudal plane, with loss of an interface between it and the pectoralis major muscle, with some hyperdense linear and nodular areas inside, as well as two 9-mm ipsilateral axillary lymphadenopathies (Fig. ). The patient underwent a right total mastectomy. The postoperative period was uneventful; the patient recovered well and she went home after 3 days of hospitalization. The pathology results showed a solid, multilobed, and heterogeneous tumor 4,857 g in weight and 40.2 × 36.3 × 15 cm in size. Moreover there was a skin ulceration of 8 cm in the nipple (Fig. ). Microscopically, the sections showed a benign mixed fibroepithelial neoplasm with areas of fibroadenoma and benign phyllodes tumor, as well as tumor-free surgical margins (Fig. , ). During follow-up, there has been no evidence of local relapse or distant metastases to date.


A 50-year-old man presented to our hospital in June 2018 with a two-month history of progressive fatigue. Past medical history was significant for diabetes mellitus, which was well controlled with antidiabetic medications. Examination revealed marked pallor and no HSM or lymphadenopathy. Complete hemogram revealed pancytopenia (hemoglobin, 6 g/dL; white cell counts, 3.25×109/L; and platelets, 78×109/L) without any atypical cells. BM aspiration (BMA) performed at an outside hospital in May 2018 was a dry tap. BM biopsy revealed extensive reticulin fibrosis [World Health Organization (WHO) grade 2] without any blasts (). He was managed symptomatically with blood transfusions. In July 2018, peripheral blood (PB) examination showed a left shift with 13% blasts. He did not have eosinophilia or basophilia. Moreover, BMA performed in July 2018 was a dry tap. BM biopsy revealed dense reticulin fibrosis (WHO grade 2) admixed with blasts. Findings of PB flow cytometry (FCM) were consistent with the diagnosis of precursor B-cell ALL (Pre-B ALL; ). BM cytogenetics could not be performed due to dry tap. Reverse transcription polymerase chain reaction (RT-PCR) using PB was positive for the BCR-ABL transcript (p190), whereas JAK-2, CALR, and MPL mutations were negative. He was treated with the European Working Group for Adult ALL (EWALL) protocol for Philadelphia-positive ALL (Ph+-ALL) along with imatinib. EOI BMA and biopsy (day-33) were in morphological remission, with significant resolution of BM fibrosis (WHO grade 0–1). Complete molecular remission was achieved at 3 months. However, the disease relapsed after 1 year, and he died 2 months after relapse.


A 14-year-old boy presented to our hospital in May 2019 with complaints of high-grade fever and epistaxis for 1-month duration. Examination revealed marked pallor and no HSM or lymphadenopathy. Complete hemogram revealed pancytopenia (hemoglobin, 5 g/dL; white cell counts, 0.8×109/L; and platelets, 20×109/L). In March 2019, BMA performed at an outside hospital was a dry tap. BM biopsy revealed extensive reticulin fibrosis (WHO grade 3) without any immature cells or granuloma. In addition, in May 2019, repeat BMA performed at our hospital was a dry tap. BM biopsy revealed reticulin fibrosis (WHO grade II) with occasional immature cells [CD34+ and CD117+ on immuno-histochemistry (IHC)]. However, immature cells could not be characterized further by IHC. The patient was closely followed up and supported with transfusions when indicated. Fever work-up was inconclusive. The vitamin-D and parathyroid hormone levels were within normal limits, and antinuclear antibodies were absent. In June 2019, PB findings were unremarkable except for occasional blasts, FCM of PB was inconclusive, and BMA was a dry tap. BM biopsy revealed dense fibrosis (WHO grade 2), dysplastic megakaryocytes, and occasional blasts. The patient was managed symptomatically with transfusion support. In August 2019, BM was successfully aspirated from the sternum. FCM of BMA revealed early T-precursor ALL (ETP-ALL, ). BM cytogenetic analysis revealed a normal male karyotype. He was treated with the Berlin–Frankfurt–Münster (BFM)-95 protocol for ALL. EOI BMA and biopsy on day 33 revealed morphological remission and significant resolution of BM fibrosis (WHO grade 0–1). He was continued on chemotherapy and is currently in the maintenance phase.


A 25-year-old man was admitted to the hospital for liver transplantation for multiple hepatic masses that were incidentally discovered by imaging. He had previously been diagnosed with CHF on liver biopsy 14 years ago, and had received transjugular intrahepatic portosystemic shunt for portal hypertension at that time. He had no history of metabolic syndrome, alcohol intake or prior intake of androgens and his body mass index was 25.2 kg/m2. There was no evidence of other abnormalities associated with CHF, and there was no remarkable family history. Preoperative liver function tests were as follows: aspartate aminotransferase 35 U/L, alanine aminotransferase 29 U/L, gamma-glutamyl transferase 19 U/L, alkaline phosphatase 79 U/L, total bilirubin 4.2 mg/dL, and direct bilirubin 1.3 mg/dL. Serological tests for hepatitis B and C virus were negative. On computed tomography, there were multiple fat-containing nodules in both hepatic lobes.
The liver weighed 1,242 g, and sections revealed nine discrete tan-to-yellow nodules in both lobes, measuring 9.5 × 6.5 × 7.5 cm in the largest one. The background liver was firm with a reticular appearance (). On microscopic examination, the background liver showed the typical histological features of CHF: broad fibrous bands rimmed by small bile ductular structures with ductal plate malformation patterns (). The pathological findings of the nine nodules are summarized in and and . Six nodules were diagnosed as H-HCAs and the three smaller nodules as large regenerative nodule (LRN) or focal nodular hyperplasia (FNH)-like nodules, based on the histo-pathological and immunohistochemical characteristics. Notably, although LFABP loss was seen in all six H-HCAs, nodules #1, #2, and #9 showed only patchy mild steatosis, and nodules #1 and #2 also demonstrated focal peliosis and mild ductular reaction. The remaining 4 H-HCAs demonstrated diffuse steatosis. On follow-up, the patient is currently well without evidence of recurrence.


A 47-year-old male with known chronic obstructive pulmonary disorder presented to the emergency department with cramping and severe abdominal pain (visual analog scale: 10/10), which was aggravated by physical activity and breathing. He also had nausea and was vomiting. He had been experiencing these symptoms for a day. Upon clinical examination, his abdomen was distended and hard to palpate with epigastric tenderness. His blood pressure was 100/60 mm Hg, SpO2 97, pulse 150/min, and body temperature 37.2°C. His general status was moderate, and he was orientated and cooperative.
Laboratory investigations revealed a highly lipemic sample (Fig. ) with serum triglyceride (TG) level of 7,520 mg/dL (84.9 mmol/L) and total cholesterol of 870 mg/dL (22.5 mmol/L). The leukocyte count was 11.4 G/L, neutrophil ratio was 91.3%, C-reactive protein 161.1 mg/L, serum amylase levels 152 U/L, and serum lipase 507 U/L. Coagulation and biochemistry parameters could not be tested due to lipemic stimulation (lactescent serum; Fig. ).
Abdominal ultrasound detected mild hepatomegaly with increased liver echogenicity and the pancreas could not be visualized because it was obscured by bowel gas. Abdominal X-ray was normal. Contrast-enhanced computer tomography (CT) scan of the abdomen was suggestive for acute pancreatitis (AP) with peripancreatic adipose tissue infiltration, necrosis of the pancreatic tail, and with modified CT severity index of 10 (Fig. ).
The patient's Bedside Index of Severity in Acute Pancreatitis (BISAP) score was 1 at the initial evaluation. The patient was treated with severe HTG-induced AP (according to the revised Atlanta classification 2012) []. Due to the high mortality rate of HTG-induced AP, plasmapheresis was performed immediately. After 1 session of plasma exchange, his TG level decreased to 190.43 mg/dL (2.15 mmol/L) and his cholesterol level to 212.7 mg/dL (5.5 mmol/L). Afterwards, enteral feeding, aggressive fluid resuscitation, and pain management were administered, which resulted in the improvement of the patient's condition. Statin, fibrate, and low-molecular weight heparin were administered as well. On day 6, the patient experienced severe dyspnea, and his inflammatory parameters in laboratory findings started to increase again. The X-ray revealed hydrothorax (HTX), and a large amount of fluid was visible on the left side. On the following day, the patient became febrile (38°C); therefore, antibiotic therapy was initiated, but his clinical condition deteriorated regardless: his blood pressure became 114/74 mm Hg, his oxygen saturation decreased to 92%, and his BISAP score increased to 3. Control abdominal CT scan showed necrotizing pancreatitis, HTX, and a progressed level of abdominal fluids (modified CTSI: 10). On day 8, the patient was referred to the Sub-Intensive Care Unit for further care. On day 11, due to his stable vital parameters and regression of the abdominal fluid collection, he was transferred back to the Department of Gastroenterology. In the following days, his general status improved: inflammatory parameters in the lab regressed, he became permanently afebrile, and control CT scans showed a significant regression of abdominal fluid collection and complete remission of HTX (modified CTSI: 6). After 29 days of hospitalization, the patient was discharged without symptoms and in good general condition, with fibrate and statin medication. However, PCSK9 inhibitor was not initiated in this case, but it is inevitable that in the event of unsuccessful statin and fibrate medication it should be implemented in the therapeutic regimen.
AP is one of the most important diseases that should be considered in the differential diagnosis in patients presenting to emergency departments with abdominal pain. AP can be induced by several different etiological factors. The third most common cause of AP is elevated TG levels. HTG leads to the most severe form of pancreatitis with a high mortality rate among all precipitating factors []. Though its exact pathogenesis remains unclear, it is known that the metabolites of TGs may induce direct and indirect cellular damages causing pancreatitis and, eventually, necrosis [].
Unfortunately, there is no guideline available for managing HTG-induced AP. Rapid reduction of serum TG and its metabolites might be effective in preventing organ damages and should be taken into consideration []. Early diagnosis of these patients at the emergency department is crucially important since early management of HTG-induced AP is inevitable for the reduction of severity and mortality.


A 41-year-old male patient visited our hospital with the main complaint of an asymptomatic skin lesion beneath the third left nail of the hand. Six months prior, the patient presented with discoloration on the third left nail, a unilateral lesion beneath the nail, and a growing skin lesion without symptoms. There was no relevant history or family history, and there were no abnormalities in the laboratory findings. The patient's lesion was observed in the third left finger with yellowish discoloration and hypertrophy of the nail accompanied by distal onycholysis and an erythematous nodule on the proximal nail fold (). A punch biopsy was performed under the nail. The biopsy results indicated the presence of several small and dilated blood vessels between irregularly arranged proliferated spindle cells, a hypercellular area with several spindle cells, and a hypocellular area with several collagen fibers. Immunohistochemical staining was performed, which showed that the tumor cells were positive for CD34, Bcl-2, and CD99 and negative for S-100, FactorXIIIa, and smooth muscle action (SMA) (). Based on the clinical features and histologic findings, the patient was diagnosed with a subungual SFT. The patient was transferred to the department of plastic surgery for surgical treatment. We received the patient's consent form about publishing all photographic materials.


A 70-year-old man presented with multiple erythematous to violaceous papules and excoriations on both lower extremities for two weeks. The lesions started as pea-sized papules on both ankles and had spread to the entire lower extremities. The nature of excoriation occasionally bled and had been macerated. Subsequent oral steroid and empiric antibiotics failed to alleviate the lesions. The patient had no medical condition or family history of skin cancer and was otherwise in good health. Vital signs were stable and numerous non-tender 1 to 2 cm sized papules overlying erythema with pitting edema were present on physical examination ().
Laboratory results revealed severe leukocytosis (63,500/mm3), elevated erythrocyte sedimentary rate (41 mm/h), elevated C-reactive protein (24.3 mg/L) with high turnover markers including alkaline phosphatase (458 U/L) and lactate dehydrogenase (1,067 U/L). Splenomegaly and hepatomegaly were observed on enhanced abdominal computed tomography. Peripheral blood smear revealed major percentages of neutrophils with all stages of neutrophilic precursor cells including promyelocytes, myelocytes and metamyelocytes (). Counting of peripheral blood smear revealed over 90% of segmental neutrophils with 4.6% of neutrophilic precursor cells. Bone marrow biopsy remarked hypercellular nature with abundant neutrophils and scanty erythroid cells and megakaryocytes (). Skin biopsy performed on the latest erupted lesion revealed diffuse dermal infiltration of inhomogeneous neutrophils with pustules on scanning magnification (). Higher magnification showed variable sizes and shapes in nucleus with both mature and immature neutrophilic components. Atypia and mitotic figures were also pronounced (). Bizarrely large cells with multiple segmented bands are dispersed with heavy infiltration of neutrophils (). Staining of myeloperoxidase was consistent with the leukemic nature of the infiltrates (). The patient was initially diagnosed with chronic myelogenous leukemia. However, evaluation of CSF3R gene mutation was performed when negative results were confirmed in BCL-ABR, JAK2, and V617F tests. The pathologic variant was observed at the 1,853th base-sequence, where cytosine was transferred to thymine, leading to subsequent substitution of threonine to isoleucine ().
Oral hydroxyurea at a dose of 500mg twice a day and daily cefazolin 2 g was given to the patient. After one week, both the leukocyte count and skin lesions improved. Antibiotics were withdrawn and hydroxyurea was reduced to half of the initial dosage. After two weeks, the previously improved skin lesions aggravated in response to marked increase of leukocyte count (). Hydroxyurea was raised to the initial dosage, however, cutaneous lesions and leukocytosis showed no improvements (). Dosage of hydroxyurea was doubled for two weeks and the severity of skin lesion mitigated as the level of leukocytosis decreased (). After two months, the patient was administered with interferon alpha and no further drastic fluctuation in both skin lesion and leukocyte counts were observed since then. All photographic materials for the purpose of publication were displayed with the patient's consent.


A 28-year-old male patient presented with persistent scrotal erythema and inguinal striae of 6 months duration. He had a previous diagnosis of onychomycosis and tinea cruris treated with itraconazole. He had employed a topical combination cream of betamethasone, gentamicin, and clotrimazole for 14 months in the inguinal region with suspension 6 months before evaluation. He referred erythema, warmth, and dysesthesia. On examination, the patient had scrotal involvement and atrophic inguinal striae. The patient was treated with pregabalin 150 mg at night for 3 months with complete remission.


A 53-year-old male patient presented with scrotal erythema of 2 months duration. He had a previous diagnosis in another institution of contact dermatitis in the area. He had employed topical hydrocortisone and other non-specified topical treatments for 2 months. He referred erythema, a burning sensation, and pain in the scrotum. Clinical examination revealed bright-red erythema in the scrotum. No clinical signs or suggestive symptoms of contact dermatitis were noted. The patient had suspended topical treatment 2 months before evaluation. Because of the low-suspicion of contact dermatitis, patch test was considered not appropriate at the time. The patient was treated with pregabalin 150 mg at night for 2 months with complete remission.


A 63-year-old male patient presented with scrotal erythema of 3 months duration. He had a previous diagnosis of tinea cruris and had employed a combination cream of betamethasone, gentamicin, and clotrimazole for 1 month. He referred erythema, a burning sensation, and warmth. Clinical examination revealed scrotal erythema. Treatment with pregabalin 150 mg at night for 1 month resulted in complete remission.


A 57-year-old male patient presented with scrotal and penile erythema of 3 months duration. He had a history of combination cream (betamethasone, gentamicin, and clotrimazole) used for 2 months (). He referred a 3-month symptomatology of erythema, dysesthesia, pain and warmth. Pregabalin 150 mg nightly and doxycycline 100 mg every 24-hour resulted in complete resolution after 1-month of treatment ().


A 53-year-old male patient presented with scrotal erythema of 3 months duration. He had a previous diagnosis of tinea cruris and had employed desonide and a combination cream of betamethasone/gentamicin/clotrimazole for 2 months. He referred a 3-month symptomatology of erythema, warmth, pain, a burning sensation, pruritus and dysesthesia. Pregabalin 150 mg nightly was prescribed. At 1-month follow-up the patient referred 70% symptomatology improvement with slight drowsiness. After 3 months of therapy, the patient referred complete remission.


A 68-year-old Thai man presented with intermittent right upper quadrant pain, nausea and vomiting for 12 months. Two days prior to admission, he had a fever, right upper quadrant pain and jaundice. His medical histories were essential hypertension and a long history of chronic obstructive pulmonary disease (COPD). His current medications were enalapril 5 mg, two times a day; salmeterol/fluticasone propionate (50/250), one puff twice a day and ipratropium bromide/fenoterol two puffs prn. On physical examination, his body temperature was 37.8°C, pulse rate was 99 beats per minute, blood pressure was 140/80 mm Hg and respiratory rate was 18 breaths per minute. He had jaundice with icteric sclera. Abdominal examination revealed mild abdominal distension, normal bowel sound and moderate tenderness at right upper quadrant; liver and spleen were not palpable.


A 50-year-old woman presented with abnormal liver function tests after routine bloodwork. Her history was notable for NF1, type 2 diabetes mellitus and dyslipidemia. She had a previous surgical history of laparoscopic cholecystectomy, appendectomy, hysterectomy and left salpingo-oophorectomy. Labs showed an AST of 68 IU/l, ALT of 95 IU/l, alkaline phosphatase of 394 IU/l and total bilirubin of 1.1 mg/dl.
Initial ultrasound revealed significant intrahepatic and extrahepatic biliary dilation. Fatty liver was first considered due to her obesity. Despite over 40 pounds of weight loss over the next 2 months, labs continued to rise, and she had developed pruritis. She had an AST of 343 IU/l, ALT of 295 IU/l, alkaline phosphatase of 590 IU/l and total bilirubin of 1.6 mg/dl. Abdominal CT revealed worsening dilation of the main pancreatic duct to 6 mm and common bile duct dilation to 25 mm suggesting an ampullary soft tissue mass protruding into the duodenum.
The patient underwent esophagogastroduodenoscopy (EGD) and endoscopic ultrasound with fine-needle aspiration (FNA), revealing a 21 × 17 mm polypoid ampullary mass causing biliary and pancreatic duct obstruction (). FNA biopsy initially came back as polypoid duodenitis. The patient was referred for endoscopic submucosal dissection where a 20-mm submucosal mass was found in the papilla major and removed. Pathology came back for well-differentiated, low-grade G1 (based on 2010 WHO Criteria) 1.1-cm ampullary neuroendocrine tumor staining with a positive posterior margin ().
At this point, the patient was presented with two options: lifelong surveillance with EGD or Whipple procedure. Ultimately, the patient elected for Whipple. An umbilical hernia was discovered during her preoperative office visit that she opted to have repaired simultaneously.
Intraoperatively, two small nodules were found and removed from the surface of the liver. The frozen section came back with focal nodule hyperplasia. During resection of the proximal jejunum, a 10-mm polypoid mass was appreciated on the surface. The umbilical hernia sac was also removed.
Pathology report revealed the distant umbilical sac was positive for a 2.8-cm metastatic NET (). Four of 19 peripancreatic lymph nodes were positive for metastatic NETs (). Incidental gastrointestinal stromal tumor (GIST) turmorlets less than 1 cm in size were identified in the proximal jejunum (). There was extensive PIN Grade 1–2 found at the pancreatic neck margin and main duct intraductal pancreatic mucinous neoplasm (IPMN) with dysplasia and side branch IPMN ().
The patient’s postoperative recovery course was uneventful. Positron emission tomography/computed tomography (PET/CT) was planned, followed by routine imaging surveillance to monitor for additional metastasis.


A previously healthy 23-year-old male patient who presented to the Emergency Room with sudden onset of excruciating headache. On initial assessment, his Glasgow coma score (GCS) was 14 over 15, with equal reactive pupils bilaterally and no apparent neurological deficit.
In the peripheral hospital, brain computed tomography (CT) revealed extensive subarachnoid hemorrhage (SAH) with no significant hydrocephalus. While completing his workup in the emergency department, his level of consciousness rapidly dropped to 8 over 15 with no apparent seizure attack. He was immediately intubated for airway protection and started on sedation. The brain CT was repeated immediately to reassess for presumed aneurysm re-rupture or obstructive hydrocephalus.
The repeated image did not reveal new bleed or worsening of hydrocephalus. However, the neurosurgeon elected to insert an external ventricular drain for monitoring and preparation of transfer to another health-care facility for definitive treatment. The cerebral computed tomography angiogram (CTA) revealed a 5x4x4 mm bilobed right distal anterior cerebral artery aneurysm (DACA) (pericallosal aneurysm).
After arrival at our hospital, the patient was moving all his limbs and opening his eyes only to painful provocation. He was taken urgently to the angiography suite. The aneurysm was occluded successfully using the coiling technique, and there were no intraoperative complications ().
Postoperatively, he received standard Intensive Care Unit supportive therapy for a few days. The neurosurgery team and ICU made the patient extubated. Upon extubation, the neurological examination showed that patient eyes were closed. He did not speak spontaneously. With the painful stimulation, the patient opened his eyes. He uttered a few slurred and dysarthric words. In the next few days, he started to obeying single step commands and opened eyes spontaneously. He was awake, but the patient expressed signs of apathy. He demonstrated a lack of motivation, indifference, and a scarcity of facial and emotional expressions when interacted with a close family member during the ICU visits. No abnormal movements observed, and no grasp reflexes noticed. Over the next 3 weeks, he had spontaneous speech but diminished. He started to have a normal spontaneous movement in all 4 extremities within the context of preserved alertness.
Magnetic resonance imaging (MRI) of the brain showed infarction of the entire body of corpus callosum (). The patient underwent multiple visits to the angiography suite to check for any possibility of vasospasm; however, a repeated cerebral angiogram revealed mild vasospasm in distal anterior cerebral arteries branches ().
Clinically, the patient was not in a coma or decreased consciousness; he remained in the akinetic mutism status for 21 days. We believe that he was in akinetic mutism due to the persistent presence of apathy, abulia, lack of motivation, and emotional response, along with the initial lack of spontaneous speech with preserved wakefulness. The patient then started to gradually recover, regaining full speech first then motor function, until he had complete recovery after three months.


A 23-years-old female patient, with a history of marijuana addiction for one year, tobacco and alcohol consumption since the age of 17, three abortions before 10 weeks of gestation, previously diagnosed in May 2012, with Human Papillomavirus (HPV) infection by cervical cytology and aplastic anemia by bone marrow aspiration (BMA). She received treatment with Antithymocyte Globulin for 2 cycles (May 2012 and April 2014) and posteriorly Cyclosporin A, with subsequent liver toxicity, changing to Mycophenolate Mofetil and Danazol, with a partial response, requiring multiple hospitalizations and transfusion support, with more than 40 units of globular packages, complicating with transfusional hemosiderosis, treated with Deferasirox. In May 2015, she presented cellulitis of the right thoracic limb, with spontaneous resolution and a secondary atrophic scar. In November 2015, she began with asthenia, adynamia and fever up to 41ºC, predominantly in the evening and a nodular lesion of 3 cm diameter in the upper inner quadrant (UIQ) of the right breast, which increased in size progressively, with very painful violaceous skin lesions, evolving to confluent blisters and later ulceration with non-purulent secretion and necrosis (). She was treated with Dicloxacillin, Piperacillin / Tazobactam and Carbapenemic. In the absence of improvement, stage IIIB breast cancer was suspected, so she was referred in December 2015 to a tertiary level hospital. Upon admission, it was documented the lesion in the right breast, with bleeding nipple, as well as confluent blisters and edema on the outer side of the left thigh (Figure 1B,C). The patient had a non-reactive viral panel for HIV, HCV, HBV. A breast skin biopsy revealed a neutrophilic inflammatory infiltrate with no evidence of vasculitis (). Treatment with systemic steroid was started, with excellent response and remission of lesions. Imaging tests were performed, as chest x-ray and Thoracoabdominal computed tomography, finding hepatosplenomegaly, the final diagnosis was necrotizing Sweet’s syndrome. A month later, a new BMA was performed, finding dysplasia of more than 50% of the cells of the erythroid and granulocytic series, as well as absent megakaryocytes, diagnosing MS. The patient persisted with transfusion support with globular packages and treatment with Mycophenolic Acid. On November 2016, the patient was hospitalized for a 2-week course consistent with fever, anemia and purpuric syndrome, low cardiac output data, syncope, and acute kidney injury. BMA congruent for AML with absent megakaryocytes, 7% adult neutrophils, 2% young neutrophils, 1% lymphocytes, 77% normoblasts, 13% myeloid blasts and 58% myeloid blasts. Immunophenotyping: 32.6% HLA-DR, 29.2% CD34, 36.5% Glycophorin, 2.08% Glycophorin A/34, 28.6% CD117, 53.8% CD13, 51% CD33, 12,7% CD64, 1.9% CD7 and 31.6% CD22. Management began with anti-tumor lysis measures, hemoderivatives, and leukapheresis. Five days after admission, the patient presented respiratory failure, requiring mechanical ventilatory support, progressing to multiple organ failure culminating in death, with final diagnoses of AML subtype M2, aplastic anemia and Sweet’s Syndrome.


A 23-year-old woman presented with three days history of odynophagia and neck pain, exacerbated by food intake and neck movement. There was no history of strenuous activity or trauma. She had no fever, neck swelling, cough, hoarseness, shortness of breath or vomiting. There was no significant medical or surgical history.
On general examination, the patient was afebrile. The neck pain was aggravated on passive neck movements towards all directions. The paracervical region was tender on palpation. There was no neck swelling yet a two-finger-breath trismus was present. Intraoral examination was unremarkable.
Flexible nasopharyngolaryngoscopy revealed a bulging posterior pharyngeal wall from the level of nasopharynx to oropharynx. The overlying mucosa appeared normal. Leukocyte count was normal. Erythrocyte sedimentation rate (ESR) was raised to 40 mm/hr. A provisional diagnosis of retropharyngeal abscess was made.
Due to the initial suspicion of foreign body in the throat by the Emergency Department, a cervical X-ray was performed that showed loss of cervical lordosis with an abnormal prevertebral thickening seen at C1 and C2 levels (). Then, we proceeded with computed tomography (CT) of the neck, which revealed a focal prevertebral soft tissue thickening at C1 and C2 levels measuring one cm in full thickness ( and ). The calcification extended into the atlantodental joint space. There was no rim-enhancing collection or air pockets at the prevertebral or retropharyngeal space to suggest an abscess. The diagnosis was then revised to RCT.
Our patient received adequate analgesia (paracetamol and tramadol) in addition to empirical antibiotics (cefuroxime and metronidazole) for presumed retropharyngeal abscess despite the impression of acute RCT obtained from the CT scan. The antibiotics were initiated prior to obtaining CT scan results, and maintained keeping in view of the infective causes as the commoner etiology of such presentations. Her symptoms improved gradually and resolved five days after the admission.
Two months later, lateral cervical radiograph and CT scans were repeated (, and ), which showed resolution of retropharyngeal/prevertebral soft tissue edema with minimal residual soft tissue calcification anterior to the odontoid process. The ESR reduced to 20 mm/hr, indicating recovery.


A 38-year-old lady presented with gum bleeding of 3 days duration, two years back. On evaluation, she had isolated thrombocytopenia (platelet count-8000 cells/μL). In view of large platelets with normal morphology on peripheral smear, the patient was diagnosed as immune thrombocytopenic purpura (ITP) and managed with intravenous methylprednisolone (1000 mg daily for three days) followed by oral prednisolone (1 mg/kg) as maintenance therapy. She initially responded to therapy (platelet count increased to 50 × 109/L), however, developed severe thrombocytopenia upon gradual tapering of prednisolone (platelet count 9 × 109/L) requiring intravenous immunoglobulin (1 gm/kg; single dose), and hiking up of oral prednisolone (1 mg/kg).
One month later, she relapsed again following steroid taper and was initiated on eltrombopag (50 mg once daily) and thereafter attained partial remission with platelet count ranging from 30–50 × 109/L. One year later, she had unprovoked distal deep vein thrombosis (DVT) of the right lower limb. In background of thrombocytopenia, possibility of systemic lupus erythematosus was considered, however, her repeat ANA, anti-ds DNA were negative, complement levels (C3/C4) were within normal limit, but she had detectable aCL [IgM-negative, IgG-28 GPL (>15)] and anti-β2GP1 [IgM-negative, IgG-38 SGU (>20)] antibodies while LA was negative. In absence of obstetrical morbidity and low titres of antiphospholipid antibodies (aPLs), the patient was managed with an oral anticoagulant (OAC) with advice for close follow-up- However, she discontinued OAC after 6 months and was lost to follow-up.
She now presented with complaints of breathlessness and reduced urine output of five days duration. She denied a history of fever, cough with expectoration, hemoptysis, orthopnea, paroxysmal dyspnea, palpitations, chest pain, gravelluria, haematuria, frothuria, intake of alternative medications, loose frequent stools, and bloody diarrhoea. Clinically, she had pallor, hypertension (BP-170/100 mmHg) and bilateral infra-scapular crepitations on chest auscultation. Investigations revealed hemoglobin - 8.3 gm/dL, leukocyte count - 8000 cells/mm3, and platelet count- 21000 cells/microL, blood urea - 131 mg/dL, creatinine - 9.7 mg/dL, with evidence of micro-angiopathic hemolysis on peripheral blood smear. Her urine examination showed dysmorphic erythrocytes without proteinuria. Troponin T was negative, 2D-echocardiography was normal, chest X-Ray suggested pulmonary edema. Ultrasonography showed bilateral normal-sized kidneys with the normal flow in renal vessels on renal doppler. Her repeat ANA, anti-ds DNA were again negative and complement levels (C3/C4) were within normal limits, however, this time she had triple positivity for aPLs with significant titers [aCL: IgM-Negative, IgG-119 GPL, anti-β2GP1: IgM-Negative, IgG-163 SGU, LA: Positive] making a provisional diagnosis of primary APS. To ascertain the etiology of rapidly progressive renal failure, she underwent renal biopsy (after correction of thrombocytopenia with platelet transfusions) which showed 16 glomeruli, out of which 4 were morphologically normal, 8 glomeruli showed endothelial swelling while remaining 4 glomeruli had extensive subendothelial widening and thrombotic occlusion of capillary lumen, with mild interstitial inflammation and edema []. Two arteries had extensive endothelial swelling with near obliteration of the lumen and fragmented erythrocytes in the intimal layer suggesting florid glomerular and vascular thrombotic microangiopathy (TMA) confirming APSN. Direct immunofluorescence was negative for immunoglobulins and complements. She was managed with plasmapheresis (40 ml/kg-5 sessions), intravenous methylprednisolone (1000 mg daily for 3 days) followed by oral prednisolone (1 mg/kg), unfractionated heparin and alternate day HD. She became nonoliguric, however, had worsening dyspnea on day 7 of admission. Pulmonary CT angiography revealed subsegmental pulmonary thromboembolism which was managed conservatively. The further hospital stay was complicated by refractory septic shock requiring empirical antibiotics, inotropes, and ventilatory support. however, She succumbed to her illness on day 11 of admission.


A 27-year-old male presented with penile swelling and difficulty in passing urine for 2 months. Physical examination showed a 5 cm × 2 cm palpable firm mass at the ventral aspect of the distal penis. Magnetic resonance imaging showed a 4.9 cm × 1.8 cm × 1.5 cm, well-defined lobulated mass lesion in the penile region involving the urethra and the corpus spongiosum, compressing the surrounding corpus cavernosum. It was hyperintense on T2-weighted imaging and hypointense on T1-weighted imaging with patchy irregular peripheral enhancement on contrast scan. The patient underwent a cystourethroscopy, which showed a pedunculated mass arising from the right half of bulbar and penile urethra almost obliterating the lumen. The biopsy revealed a small, round cell tumor. 18F-fluorodeoxyglucose whole-body positron emission tomography–computed tomography (PET-CT) scan [] showed hypermetabolic lesion involving the entire length of the penile urethra up to bulbous urethra with a predominance on the right side and no uptake in lymph nodes or distant organs.
After a discussion within the tumor board, the patient was offered total penectomy and perineal urethrostomy with adjuvant chemotherapy. Groin lymph node dissection was not performed as there was no evidence to support its role. Histopathological examination of total penectomy specimen showed [Figure 1b] sheets of small, round, blue cells with scanty cytoplasm without any nuclear molding, few tumor rosettes, and many mitotic figures. On immunohistochemistry (IHC, Figure 1c and d), cells expressed vimentin, Fli1, NKX 2.2, and mic-2. They were immunonegative for CK, EMA, CK7, CK20, p63, Gata3, chromogranin A, synaptophysin, CD56, Melan-A, HMB45, and S-100p. The fluorescence in situ hybridization (FISH) analysis showed a rearrangement of the EWSR1 gene locus in 96% of the tumor cells [Figure 1e; done with ZytoLight SPEC EWSR1 Dual Color Breakapart Probe Kit, analyzed on an Olympus BX51 fluorescent microscope using Applied Spectral Imaging Software]. The tumor was seen as a submucosal periurethral polypoidal mass infiltrating the corpus spongiosum. The margins of resection were free. Based on these findings, the diagnosis of PNET of the penile urethra was made. Postoperatively, the patient received 5 cycles of adjuvant chemotherapy (Injection etoposide 180 mg days 1, 2, 3, 4, and 5 and injection ifosfamide 3000 mg days 1, 2, 3, 4, and 5) and 3 cycles of VAC. This treatment was completed without dose modifications on the scheduled days. Follow-up studies including clinical examination and PET-CT scan were normal at 6th and 12th months. The patient is currently disease free at 12 months.


A 37-year old male injecting drug user (IDU) with recurrent biological PVE resided in a cardiac rehabilitation clinic after valve re-replacement surgery for an episode of Enterococcus faecalis and Candida dubliniensis endocarditis. Two days after completion of his six-week antibiotic treatment with intravenous amoxicillin/clavulanic acid and whilst still under isavuconazole treatment, he developed fever accompanied by elevated inflammatory markers. Subsequently, two blood culture bottles yielded growth of P. pnomenusa drawn simultaneously from his peripherally inserted central venous catheter (PICC) and from his elbow vein. The patient was started on empirical treatment with tigecycline. Upon suspicion of a central venous catheter-associated bloodstream infection (CLABSI), the patient was readmitted to the University Hospital of Zurich on day 6 of the current episode. Blood cultures drawn upon admission showed a marked difference in time to positivity of 9h between the PICC (14 hours) and the peripherally drawn blood cultures (23 hours), indicative of CLABSI. The central line was removed immediately without showing any growth microbiologically. Initial testing demonstrated susceptibility towards piperacillin/tazobactam but resistance to tigecycline when interpreted according to Pseudomonas clinical breakpoints. Accordingly, therapy was switched to piperacillin/tazobactam on day 8 of the current episode. Three days after initiation of targeted therapy, a new PICC was inserted until novel blood cultures on day 22 yielded growth of P. pnomenusa again, which led to another change of the PICC. At the same time, transesophageal echocardiography (TOE) demonstrated filiform, floating structures on the central catheter without valve vegetation, whereas the positron emission computed tomography (PET/CT) did neither show a metabolic activity of the prosthetic valve nor of the catheter tip. A small echo-dense structure at the anterior pocket of the annulus had already been described in earlier investigations. Based on suggestive clinical and microbiological criteria (fever, persistent positive blood cultures not meeting major criteria, predisposing heart condition and IDU) a treatment duration of six weeks for possible PVE was determined. After a three-week course of piperacillin/tazobactam, therapy was switched to trimethoprim/sulfamethoxazole. To date, P. pnomenusa was not isolated in the patient again.


A 17-year-old obese female with a nonsignificant past medical history presented to the emergency room (ER) because of fever, cough, and shortness of breath. Onset 7 days before admission with ungraded fever and cough, was aggravated by dyspnea, which prompted her to call the emergency medical services (EMS). The EMS reported that the patient’s oxygen saturation was in the low 80s, so she was placed on a non-rebreather mask (NRM) and transferred to the hospital. Upon arrival in the ER, the patient looked lethargic, in severe respiratory distress, and could only speak two to three words per sentence. On arrival, vitals were as follows: blood pressure (BP) 98/69, pulse 140, respiratory rate 30, saturation 98% on NRM, and temperature of 38.4 °C. On physical examination, there were fine crepitations in both lung fields. Real-time polymerase chain reaction (PCR) for COVID-19 was performed, which came back positive. Chest X-ray showed patchy bilateral opacities (). Other laboratory studies revealed white blood cell (WBC): 13.7 × 103/µL, hemoglobin (Hb): 12.5g/dL, erythrocyte sedimentation rate (ESR): 95 mm/h, D-dimer: 0.72 µg/mL, C-reactive protein (CRP): 17.1 mg/dL, ferritin: 251.2 ng/mL, blood urea nitrogen (BUN): 6mg/dL and creatinine: 0.5mg/dL. The patient was admitted to the pediatric intensive care unit (ICU); the NRM was switched to high-flow nasal cannula (HFNC) (45 L per minute (LPM) at 50% FiO2) and was started on hydroxychloroquine, azithromycin, vitamin C, and zinc, which were standard of care at the time. Remdesivir was requested from the Department of Health but was not readily available due to limited supply. On day 2 of admission, her condition worsened, with a respiratory rate in the 40 s, severe respiratory distress with the use of accessory muscles. She was then placed on a non-invasive positive pressure ventilation machine on continuous positive airway pressure (CPAP) mode to improve the oxygenation. The chest X-ray also showed worsening in her lung infiltrates (). At this point, she had a very low threshold for intubation due to her worsening clinical state. This prompted the medical team to discuss other options available to manage the patient, and it was finally decided to start on immunomodulators for compassionate use. So the patient was started on baricitinib 4 mg oral dose, given daily for 5 days.
On day 3, there was a marked improvement in the patient’s clinical status, no longer in respiratory distress, with a respiratory rate 18 - 22; she looked comfortable, not using any accessory muscles. Vitals showed BP 116/55 mm Hg, heart rate (HR) 97, temperature of 37.1 °C. Oxygen delivery mode was deescalated from CPAP back to HFNC 50 LPM and 50% FiO2, which was further deescalated to Venturi mask, and then nasal cannula. Repeat chest X-ray showed a marked improvement in the opacities.
By day 6 of admission, the patient was comfortable with ambient air, saturating 98%. The patchy opacities on the chest X-ray was resolving significantly; and the inflammatory markers are improved (). She was discharged home on day 8 and scheduled for follow-up in the pediatric clinic.


A 67-year-old man presented to our facility on April 2, 2020 with fever, cough, lethargy, and worsening hallucinations. Past medical history was significant for hypertension, type 2 diabetes mellitus, and schizoaffective disorder treated with lithium and carbamazepine. Family history was noncontributory. The patient resided in a group home with a known underlying mild intellectual disability. He denied sick contacts or recent travels. Social history was unremarkable for any alcohol or recreational drug use.
On physical examination, the patient was febrile (38.2 °C) with a blood pressure of 139/85 mm Hg, heart rate of 95 beats/min, and respiration rate of 16 breaths/min. Oxygen saturation was 92% on room air. Pulmonary examination revealed normal effort and breath sounds without wheezes or rales. On neurologic examination, the patient was alert, fully orientated to self, time, and place with no focal neurologic deficits. Laboratory workup included a complete blood count, complete metabolic panel, blood culture and urinalysis, all of which were unremarkable. Nasopharyngeal swab polymerase chain reaction (PCR) for SARS-CoV-2 was positive. A chest X-ray and a computed tomography of the head without contrast were unrevealing. The patient was treated with hydroxychloroquine, 200 mg twice a day for 4 days. Oxygen saturation was monitored throughout the hospitalization with no evidence of hypoxia. He was discharged in a stable condition 4 days after initial presentation. Follow-up telehealth evaluation the following week reported generalized weakness and a low-grade fever, but otherwise no new neurological symptoms.
Three weeks following the initial presentation, the patient experienced worsening lethargy in the setting of decreased oral intake, severe encephalopathy, and impaired balance. The rest of the review of systems was unrevealing.
Initial neurological evaluation revealed severe encephalopathy with a Glascow coma score of 11. Patient was awake but inconsistently oriented and unable to follow complex commands. Pupils were equal, round, and reactive to light. Extraocular muscles were intact and no nystagmus was noted. The remaining of the neurological examination was limited. He was found to be hypothermic (33.9 °C). The hypothermia resolved with warmed intravenous fluids and warming blankets, but the patient remained severely encephalopathic. The differential diagnosis for altered mental status included metabolic derangements, toxicity from psychiatric medications in the context of dehydration, and late neurologic manifestation of SARS-CoV-2.
Laboratory workup revealed a white blood cell count (WBC) of 16.21 × 103/µL, hemoglobin of 14.4 g/dL, and serum glucose of 131 mg/dL. Complete metabolic panel was within normal range. Lithium and carbamazepine levels were mildly elevated to 1.5 mmol/L and 12.9 mg/L, respectively, but were dose-adjusted, monitored, and subsequently found to be within therapeutic range. No WBCs or nitrates were detected on urinalysis. Cerebrospinal fluid (CSF) analysis revealed a clear and colorless fluid with a mildly elevated protein count of 76 mg/dL and a WBC count of 3/µL (all lymphocytes). Complete meningoencephalitis viral PCR panel was negative. SARS-CoV-2 PCR testing was not performed on the CSF. An electroencephalogram revealed diffuse slowing consistent with mild encephalopathy. No epileptiform discharges or electrographic seizures were noted. Ten days after admission, patient exhibited decreased inspiratory effort and breath sounds at the lung bases. SARS-CoV-2 was detected in a nasopharyngeal swab by PCR. The patient gradually improved with supportive care, and gradually returned back to his neurologic baseline over the course of several weeks.


Our patient is a 47-year-old gentleman, never-smoker, who presented with the development of right-sided facial numbness in the setting of an enlarging neck mass, which he noticed approximately 7–8 months prior to initial presentation. He was not experiencing pain, and no lymphadenopathy was noted. A CT scan showed a non-specific mass in the right parotid gland, and MRI confirmed a complex cystic and solid mass in the deep lobe, which appeared sharply defined relative to the surrounding parotid (). No regional adenopathy was identified.
Fine needle aspiration of the mass revealed atypical squamous cells in the setting of a cystic lesion, but was largely nondiagnostic. The patient underwent a right-sided parotidectomy with ipsilateral neck dissection of levels I through III. The inferior-most branches of the facial nerve were sacrificed because of adherence to tumor. Pathology of the surgical specimen showed an intermediate grade mucoepidermoid carcinoma, 1.8 cm in greatest dimension, with peripheral cystic changes (). The tumor was confined within the parotid gland and margins were negative, although the closest margin was <1 mm from tumor. Pathology did not reveal lymphovascular or perineural invasion, and zero of 31 lymph nodes were positive for tumor. The patient was staged as pT1, pN0, M0.
Post-operative radiotherapy was recommended due to intraoperative findings of suspicious tumor tracking along the facial nerve, close margins, intermediate grade, and invasion into the deep parotid lobe. A dose of 60 Gy was prescribed to be delivered in 30 fractions using intensity modulated radiation therapy. Radiotherapy was delivered to the right parotidectomy tumor bed including the facial nerve in the stylomastoid foramen and facial canal, and level Ib and II cervical lymph nodes on the right.
The patient tolerated treatment well and was followed at regular intervals. Seven years after initial diagnosis and treatment, the patient developed a nontender mass in the left parotid area, accompanied by left ear pain and pressure. Physical exam showed a firm, mobile mass in the left preauricular area adjacent to the tragus and external auditory canal. There was no lymphadenopathy. MRI revealed an enhancing mixed signal in the superficial lobe of the parotid gland: a 1 cm mass with mild exophytic extension into adjacent subcutaneous tissues (). Ultrasound revealed a hypoechoic, partially solid, partially cystic nodule in the superficial portion of the left parotid gland. Fine needle aspiration was performed and was non-conclusive, although suspicious for low-grade mucoepidermoid or ACC. Chest X-ray was normal.
The patient underwent a superficial left parotidectomy: the mass was 1.3 cm, superficial, well-encapsulated, and not adherent to the facial nerve. It was completely resected with negative margins (1 mm), and found to be well-differentiated mammary analogue secretory carcinoma. Zero of two lymph nodes were involved by tumor, and no lymphovascular invasion or perineural invasion was identified. The pathology department compared the new slides with the tumor from seven years prior, and found that the histologies were the same (). No adjuvant therapy was recommended since the second tumor was low-grade, encapsulated, small, within the superficial lobe, and completely resected. There has been no recurrence of either tumor as of August sixth, 2020.
After the re-classification of the first tumor as SC, tissue from the two tumors was submitted for fluorescence in situ hybridization (FISH) to detect possible ETV6 rearrangement at 12p13. This rearrangement was present in the 2016 specimen (), but unfortunately the 2009 specimen was decalcified and not suitable for FISH. Regardless, histopathology of the 2009 specimen supported the diagnosis of secretory carcinoma ().


We present the case of a 63-year-old female with prior percutaneous revascularizations after a single vein bypass graft to the left anterior descending artery, presented with Canadian Cardiovascular Society (CCS) Angina Grade III. Physical examination and laboratory were unremarkable. She had a history of known, severe, life-threatening anaphylactic reactions to previous iodinated dye usage leading to respiratory arrest requiring intubation (despite adequate prophylaxis with steroids and antihistamines). Given her prior history of anaphylactic reaction to iodinated contrast, progressive escalation of medical therapy without repeat angiography was recommended. However, the patient continued to experience a progressive decline in quality of life despite maximal medical therapy. Therefore, coronary and bypass graft angiography was recommended. Coronary and bypass graft angiography was performed using 24 cc of undiluted Gadodiamide (OMNISCAN) with a power injector (ACIST®) without any incidents or premedication. The patient’s coronary artery disease was essentially unchanged from her prior angiogram () and further intensification of anti-anginal therapy was recommended. Patient was monitored for 24 hours for arrhythmia and had repeat renal function in 24 hours and one-week post procedure, were within the normal limits.


A 20-year-old male patient presented with right-sided genu valgum deformity and 2 cm true shortening without DNVD. The patient had a history of trauma over the right knee 5 years ago which was managed conservatively with a splint. The patient noticed right knee deformity at the age of 16 years which progressed gradually until the age of 19 years. The deformity is non-progressive since then. The patient complained of occasional pain over knee. On examination, no tenderness over knee could be elicited and patellar tracking was normal. There was no ligamentous laxity around knee. His medical history and family history were unremarkable. The patient wanted correction of deformity and consented for surgery for same.
Specific pre-operative radiological investigations performed were standing bilateral lower limb scanogram for exact measurement of deformity (essential for bony deformities []) and magnetic resonance imaging (MRI) to evaluate both compartments of knee. () shows pre-operative scanogram. On scanogram, angles were calculated on computer software to avoid manual error and to take account of magnification on the film. Lateral distal femoral angle was 67° with knee joint line obliquity of 7° varus which is abnormal. Joint line obliquity on opposite side was 3° varus. When adjusted for joint line obliquity, genu valgum deformity turned out to be 16° at the level of distal femur. Usually, genu valgum occurs due to distal femoral malalignment and should be corrected with distal femoral osteotomy while genu varum occurs due to proximal tibial malalignment and should be corrected with high tibia osteotomy []. Medial proximal tibial angle could not be calculated accurately because of bowing of tibial shaft. Tibial bowing was considered as multiapical deformity and total deformity of tibial shaft turned out to be 6°. This gives total deformity of 22°. There was 2 cm of true shortening as well. Center of rotation of angulation (CORA) of bowing deformity of tibial shaft was found at the level of junction of upper third and middle third of shaft of tibia. There is marked mechanical axis deviation (MAD) to lateral side. Neck shaft angles were 130° on the right side and 135° on the left side. No deformity was noted in sagittal plane. MRI revealed normal lateral compartment, medial compartment and other structures of knee obviating need of diagnostic arthroscopy.
Goals of correction were to restore mechanical axis of lower limb while maintaining knee joint line obliquity (3° varus) and to achieve single-stage correction without neurovascular injury.
Total genu valgum deformity (22°) was divided into two components: Distal femoral component (16°) and tibial shaft component (6°). Surgical plan was devised for a single-stage correction utilizing medial distal femoral closing wedge osteotomy with translational osteotomy at CORA of shaft tibia. Closing wedge osteotomy was chosen over opening wedge due to higher biomechanical stability and scope of allowing early weight-bearing []. () shows planned osteotomy.
Medial distal femoral closing wedge osteotomy was done using medial approach to distal femur. Initial 2 K-wires were placed exactly parallel to knee joint line at metaphysis anteriorly and posteriorly. Perfect parallel placement of K-wires prevents flexion or extension deformity. Mediolateral diameter of osteotomy was calculated from these K-wires using subtraction method.
(Tab. 1) Size of the wedge to be resected for desired correction in mm.
Size of the wedge to be resected was calculated from trigonometric table devised by Wylie et al. []. This table has inputs in form of mediolateral diameter of osteotomy and degrees of correction required (). Once the size of wedge was calculated, the second pair of K-wires was placed in convergent fashion after measuring the medial base with scale. Blunt Hohmann retractors were placed anteriorly and posteriorly to prevent injury to quadriceps anteriorly and neurovascular structures posteriorly. Osteotomy was carried out using oscillating saw using K-wires as guide. Specific care was taken to stop osteotomy 10 mm before lateral cortex to maintain lateral bony hinge and improve inherent stability of construct. Furthermore, completion of osteotomy at posterior cortex was done with osteotome to prevent injury to neurovascular structures. Bony wedge was removed and osteotomy was closed gently. Correction was checked by interoperative c-arm imaging using cautery wire. Osteotomy was fixed using 4.5 mm locking compression plate placed on anteromedial aspect of distal femur under direct visualization.
Translational osteotomy of tibia was done at CORA followed by internal fixation with tibia interlocked nail. Specific care was taken to prevent eccentric placement of nail after osteotomy which may lead to suboptimal alignment. First, tibial entry was taken with awl and guidewire was placed centrally in tibial canal. Progressive reaming was done over guidewire which created a centralized track for nail. Under fluoroscopic control, tibial osteotomy was completed with oscillating saw. Curved Hohmann’s retractors were placed around tibia to prevent iatrogenic tissue injury. Osteotomy of fibula at same level was achieved manually with osteotomes using mini-open method. Tibia interlock nail was introduced over guidewire and locking was done. This technique ensures adequate correction of tibia valga due to central placement of a straight nail in the bone. Final alignment was checked using cautery wire method which showed perfect restoration of mechanical axis. shows immediate post-operative scanogram.


A 72-year-old man with a 2-year medical history of hypertension was referred to our clinic because of a 1-month history of progressive thunderclap headaches, visual blurriness, and nausea. His blood pressure was maintained at less than 150/90 mmHg by treatment with a beta blocker (25 mg/day of metoprolol tartrate tablets, National Medicine Standard H32025391; AstraZeneca, Cambridge, UK). The headache was described as moderate intensity, worsening in the decubitus position and occurring in the bilateral occipital regions. He reported no fever, limb weakness, or convulsions. Neurological examination was unremarkable with the exception of bilateral asymmetric papilledema, which was much worse on the right side (grade 4 in right eye and grade 2 in left eye as evaluated with the Frisén scale) ().
The results of routine laboratory tests of the blood and cerebrospinal fluid (CSF) after admission were as follows: white blood cell count, 7.66 × 109/L; red blood cell count, 4.50 × 109/L; C-reactive protein, 8.0 mg/L; activated partial thromboplastin time, 38.3 s; international normalized ratio, 1.04; glucose level in CSF, 51.12 mg/dL; protein level in CSF, 43 mg/dL; D-dimers, 0.56 µg/mL (reference range, <0.5 µg/mL); and fibrinogen, 5.77 g/L (reference range, <4.0 g/L). The lumbar puncture opening pressure was 330 mmH2O, and the CSF composition was normal. Ultrasonography of the carotid artery demonstrated intima-media thickening and one stable carotid plaque. Magnetic resonance venography (MRV) revealed poor visualization in the right transverse and sigmoid sinus, which was suspected to indicate thrombosis (). The thrombi in the right transverse sinus were directly displayed on the magnetic resonance black-blood thrombus imaging (MRBBTI) map (). The IJVs were visualized by three-dimensional computed tomography venography (3D-CTV) reconstruction, which revealed that the stenosis of the bilateral IJVs had been induced by a left lateral mass of the C1 vertebra and the right expanded carotid artery, respectively (). In addition, optical coherence tomography revealed papilledema, abnormal retinal thickness, and macular edema with greater severity in the right eye ().
The patient received an intravenous infusion of 5 BU of batroxobin (National Medicine Standard 20031074; Beijing Topixi Pharmaceutical Co., Ltd., Beijing, China) every other day for a total of three doses, a subcutaneous injection of 0.6 mL of enoxaparin sodium (National Medicine Standard H20100485; Sanofi, Paris, France) every 12 hours, and an intravenous infusion of 125 mL of 20% mannitol (National Medicine Standard H34023604; Beijing Shuanghe Pharmaceutical Co., Ltd., Beijing, China) every 8 hours to alleviate his symptoms of intracranial hypertension. His symptoms gradually decreased in severity during the treatment. At the 7-day follow-up examination, his symptoms were remarkably relieved with great improvement of the papilledema (grade 1 on Frisén scale) (). The follow-up lumbar puncture opening pressure was 185 mmH2O, and the CSF workup was normal. Moreover, both the fibrinogen level (2.07 g/L) and D-dimer level (0.43 µg/mL) had decreased to within the reference range. Compared with the baseline imaging map prior to batroxobin use, the follow-up MRBBTI and MRV on post-treatment day 7 showed remarkable shrinkage of the thrombus in situ and effective recanalization of the involved sinus ( versus (e) and (f)). Follow-up optical coherence tomography also indicated that the papilledema, retinal thickening, and macular edema had been remarkably attenuated, with a decrease in the Frisén grade from 4 to 1 ().


We report a rare case of a 32-year-old male, a known case of leprosy presented to our tertiary hospital in February 2017 with pain, swelling, burning sensation, and numbness of multiple joints. The pain started on his ankle and feet, with both hands and feet numbness 9 months ago. Joints swelling and pain gradually developed in the metacarpo-phalangeal joints, proximal inter phalangeal joints, and wrist joints with decreased hand grip (). He also had recurrent episodes of low-grade fever and large maculopapular skin lesions (approximately 5cm in diameter) which was hypopigmented, with a roughly symmetric distribution, scattered throughout the back and arms, with central hypoesthesia. He had no painful or thickened nerve roots. Tenderness over right ulnar nerve and left common peroneal nerve were present.
Laboratory investigations revealed white blood cell count of 36,800 (normal 4000–10,000) (93% neutrophils), hemoglobin of 7.9 g/dl (mean corpuscular volume of 74.6 and mean corpuscular hemoglobin of 22.3), and platelet counts of 656,000. Inflammatory markers were raised (C-reactive protein [CRP] of 61 mg/l and erythrocyte sedimentation rate [ESR] of 87 mm/1 h). Blood and urine culture did not grow any organism. Further investigation revealed negative anti-nuclear antibody, rheumatoid factor (RF), anti-cyclic citrullinated peptide (CCP), and normal complements. X-rays of hands and foot were normal.
Electromyogram and nerve conduction velocity indicated Mononeuritis multiplex affecting the nerves with preserved “H” reflexes suggestive of Hansen disease.
The patient was started on anti-leprosy drugs (dapsone, rifampicin, and clofazimine) which resulted into gradual improvement of skin lesions, but there was persistence of articular symptoms. Later during the course of treatment, he was started on oral steroid in view of CPN neuritis.
The patient had regular follow up in the outpatient clinic with infectious disease physician. Steroid was tapered slowly and the patient was improved clinically. Four months after initiation of treatment, the patient developed involvement of multiple joints including bilateral hip pain which was referred to knees (more on the left side).
Magnetic resonance imaging was suggestive of bilateral grade 2a avascular necrosis hip (Ficat-Arlet classification) () for which he underwent bilateral core decompression with delayed weight-bearing initially. After that, the patient was improved symptomatically for 6 months but later on patient started aggravation of the left hip pain which was diagnosed as the left hip arthritis () even though the patient had completed 13 months of anti-leprosy treatment till this time.
The patient was planned for total hip arthroplasty (THA) for the left hip arthritis and the patient was on anti-leprosy treatment, but intraoperative suspicious granulomatous tissue was observed and caseating necrotic fluid was present around the hip joint ();hence, planned THA was converted into staged THA with debridement and antibiotic spacer in the first stage and final hip prosthesis in the second stage.
During the first stage, debridement was carried out to remove infected and necrotic tissues as thoroughly as possible. Antibiotic (vancomycin + gentamycin) coated cement spacer along with beads was implanted for 6 weeks (). These antibiotics were chosen empirically because we were not sure about the pathology of this infected tissue around the hip joint and leprosy being involving large joint is rare. Histopathological examination confirmed hip arthritis secondarily to leprosy (). During this time, the patient had continued anti-leprosy treatment.
During the second stage, when the ESR and CRP were normal and the wound was well healed, antibiotic spacer was removed () and final hip prosthesis was implanted. The patient was advised to continue with anti-leprosy treatment for 6 months. The patients were then evaluated based on the reactivation of infection, the Harris hip score system, X-ray, ESR, and CRP ().


A 64-year-old male with a history of abdominal pain and bloody stool for several days was admitted into the clinic in June 2019. He then underwent colonoscopy biopsy, which revealed adenocarcinoma (). Abdominal contrast-enhanced computed tomography (CT) revealed that ascending colon cancer had broken through the serosa with multiple mesenteric and retroperitoneal lymph node metastases, inferior vena cava and right ureter involvement, and right kidney and right renal arteriovenous invasion (July 4, 2019) (). Based on clinical tumor-node-metastasis (cTNM), the cancer was classified as cT4N2M1. Mutation analysis revealed wildtype KRAS, NRAS, and BRAF. Immunohistochemical staining for MLH1, MSH2, MSH6, and PMS2 on the patient’s archival intestinal biopsy samples revealed loss of MLH1 and PMS2 expression (). The case was evaluated by a multidisciplinary team (MDT) that concluded that the lesions were potentially resectable and started treatment with conversion chemotherapy. Three cycles (2019-8-15, 2019-9-2, 2019-9-26) of leucovorin (400 mg/m2), 5-fluoropyrimidine (2400 mg/m2), irinotecan (180 mg/m2) and oxaliplatin (85 mg/m2) (FOLFOXIRI) resulted in stable disease (SD). Tumor response was assessed using response evaluation criteria in solid tumors (RECIST) guidelines (version1.1). In August, the patient’s serum carcinoembryonic antigen (CEA) levels also rose from 10.18ng/mL to 21.7ng/mL (normal range: 0‑3.4ng/mL). Based on the facts that immunohistochemistry showed the patient had locally confirmed MSI-H status, that he had received 1 prior line of therapy (FOLFOXIRI), and that the unsatisfactory efficacy of previous first-line regimen, the MDT developed a treatment strategy combining immunotherapy (pembrolizumab) and intensity-modulated RT to explore the potential benefit of a second intervention. After four cycles of pembrolizumab (200mg every 3 weeks) and concurrent 25 radiation treatments (metastatic lesions in retroperitoneal lymph nodes were also included in the RT field that included colon lesions, right kidney, and retroperitoneal lymph nodes), with a total dose of 45Gy (from 2019–10 to 2020–1, ) administered in conversion setting, CT scanning revealed that the patient had robust partial remission (PR) (). CEA also declined to the normal range (). Upon comprehensive assessment, the MDT concluded that the patient could undergo synchronous resection during surgery. Intraoperative dissection showed that the neoplasm was located in the ileocecal valve, involved the intestine for a cycle, and invaded the back of the right kidney parenchyma. Thus, the patient was treated with expansive radical right hemicolectomy + right nephrectomy + retroperitoneal lymphadenectomy on March 19, 2020 (). Postoperative pathological examination revealed complete primary and secondary tumor response without evidence of malignancy. Microscopic examination found only mucus but no viable cancer cells in the whole intestinal layer, perianal fat, fibrous tissue around the ureter, and lymph nodes around the vena cava (). The renal parenchyma, ureteral stump, and the distal and proximal incisions of the colon were all cancer-free. Tumor response to radiochemotherapy was complete response of Tumor Regression Grade 0 (TRG 0). No immune-related adverse events were recorded in the treatment period. The patient recovered well after surgery and received no anticancer regimens. He continues outpatient follow up 11 months postoperatively and no colon cancer recurrence has been observed through CT evaluation and CEA detection. The patient had a history of hypertension for more than 10 years and regularly took antihypertensive drugs. He had no known history of colorectal carcinoma or related gastrointestinal diseases. Appendectomy and cholecystectomy were performed for “acute appendicitis” and “cholecystolithiasis”.


A 49-year-old female patient with a 10-year history of glomerulonephritis underwent
peritoneal dialysis in November 2013 at the Department of Nephrology, HwaMei Hospital,
University of Chinese Academy of Sciences, Ningbo, Zhejiang Province, China. In addition to
continuous ambulatory peritoneal dialysis, the patient received 10 000 international units
(IU) of rHuEPO (epoetin-α; Epogen®, 3SBio, Shenyang, China) administered subcutaneously
every week from March 2014 to April 2016; and 150 mg iron polysaccharide complex capsules
administered orally every day from November 2013 up to the present time (Kremers Urban
Pharmaceuticals, Seymour, IN, USA) to maintain Hb levels at 90–113 g/l. However, in October
2016, this patient reported the major complaint of fatigue and her Hb level dropped sharply
to 67 g/l. There was no bleeding, other acute haemorrhagic events or infections.
Subsequently, a bone marrow biopsy was undertaken and there was no significant abnormality
in the bone marrow. The result of an anti-EPO antibody test using an enzyme-linked
immunosorbent assay kit (3SBio) was negative. According to her medical history and an
examination of the results described above, the dose of rHuEPO was increased to a maximum
dose of 6000 IU twice a week by subcutaneous injection. However, her Hb level did not
increase as expected and declined to 37 g/l, which required a blood transfusion to maintain
her Hb level >60 g/l. In March 2018, another bone marrow biopsy was undertaken and the
result was similar to that in 2016. Based on the test results, rHuEPO was discontinued and
she received intermittent transfusion therapy because her Hb level was 40–60 g/l.
To identify the cause of her anaemia, the patient was admitted again to the Department of
Nephrology, HwaMei Hospital on 7 December 2019. After admission, the patient received a
comprehensive evaluation including laboratory tests. The complete blood counts were as
follows: white blood cell count 8.2 × 109/l; Hb 45 g/l; haematocrit 13.1%;
platelet count 331 × 109/l; and reticulocyte count 7 × 109/l. The
levels of the components of the blood, such as iron, vitamin B12 and folic acid, were
sufficient. Based on the existing medical history and examinations, multiple myeloma,
systemic autoimmune disease, gastrointestinal bleeding and parvovirus B19 infection were
excluded. However, she needed repeated blood transfusions due to the low Hb levels.
Meanwhile, another anti-EPO antibody test was undertaken to determine the reason for her
declining Hb levels and it was positive. The patient was given the diagnosis of anti-EPO
antibody-mediated renal anaemia.
With regard to her subsequent treatment, rHuEPO was discontinued and steroids were not
administered because the patient refused to take them. The patient received 120 mg
roxadustat (FibroGen, San Francisco, CA, USA) administered orally every 72 h from 10 January
2020. After 2 months of treatment, her Hb level had reached 90 g/l and her symptoms of
fatigue had significantly improved without side-effects. The last follow-up indicated that
her Hb level was 110 g/l ().
The research protocols conformed to the provisions of the Declaration of Helsinki and were
approved by the Ethics Committee of University of Chinese Academy of Sciences, Ningbo,
Zhejiang Province, China (no. PJ-NBEY-KY-2020-183-01). The patient provided written informed
consent for publication of their data.


A 45-year-old man presented to the emergency department with acute anterior-wall MI of 8 hours duration. The patient had a history of hypertension and was a smoker. His echocardiogram showed hypokinesis in the left anterior descending (LAD) artery with an ejection fraction of 38%. The patient was thrombolysed and taken up for pharmaco-invasive PCI. His coronary angiogram showed a significant stenosis with grade IV thrombus in proximal LAD with TIMI 2 flow (). Due to high thrombus burden, stenting was deferred and the patient was put on IV eptifibatide (a glycoprotein IIb IIIa inhibitor) infusion for 18 hours followed by subcutaneous low molecular weight heparin twice daily.
Rescue PCI was planned in case the patient developed chest pain. After 5 days, his angiogram showed moderate stenosis and the thrombus in LAD was almost absent with improved TIMI 3 flow (). Since there was a significant obstruction in LAD, a direct stenting procedure with a 3 x 24 mm everolimus-eluting stent was performed and the results were optimised by post-dilation with a non-compliant balloon. The end result obtained was a TIMI 3 flow without any residual stenosis or complications.


A 53-year-old man (height, 170 cm; weight, 89 kg) was admitted to the hospital because of a 1-year history of shortness of breath after exercise. The patient was a smoker, had a history of hypertension, and was taking captopril and nimodipine. Breathing was reduced in the left lung. However, neither rhonchus nor moist rales were heard during auscultation. Chest computed tomography revealed a GEB, and the left lung was compressed by about 95% with a right mediastinal shift (). An electrocardiogram showed normal findings, and echocardiography revealed that the heart was located in the right thoracic cavity. The left ventricular diastolic function was decreased, and the left ventricular ejection fraction was 64%. Laboratory tests showed no obvious abnormalities. Arterial blood gas analysis showed the following: pH: 7.43, PaCO2: 41 mmHg, PaO2: 94 mmHg, lactate: 1.4 mmol/L, potassium: 3.6 mmol/L, sodium: 138 mmol/L, calcium: 1.10 mmol/L, FiO2: 21%, and SpO2: 98%.
On the day of surgery, the patient’s electrocardiogram, blood pressure, SpO2, and bispectral index were monitored upon entry into the operating room. A radial artery catheter and right internal jugular venous catheter were placed. The patient’s arterial blood pressure was 175/109 mmHg, heart rate was 90 beats/minute, and SpO2 was 94%. Before anesthesia, 40 µg of dexmedetomidine was infused within 10 minutes. The thoracic surgeon then created a small incision at the intersection of the sixth intercostal space and the left mid-axillary line to puncture the lung bulla and insert an indwelling catheter (14 Ga, 16 cm in length; Arrow International/Teleflex, Wayne, PA, USA) into the GEB to drain it for 15 minutes. The patient was preoxygenated with 100% oxygen at 6 L/minute for 5 minutes. Anesthesia was administered using 100 mg of propofol, 30 µg of sufentanil, and 50 mg of rocuronium. A 37-Fr right double-lumen endotracheal tube was inserted with the help of a visual laryngoscope, and single-lung ventilation was performed after proper alignment had been confirmed by fiberoptic bronchoscopy. The tidal volume was set at 5 to 6 mL/kg, the respiratory rate was 15 to 17 breaths/minute, the positive end-expiratory pressure was set at 5 cmH2O, and the inhalation:expiration ratio was 1:2. Propofol, remifentanil, and sevoflurane were used to maintain anesthesia. The intraoperative bispectral index was maintained at 40 to 50. After resecting the GEB (), one-lung ventilation was switched to two-lung ventilation and the lungs slowly expanded with a low tidal volume until complete lung re-expansion was observed. During the 3-hour surgery, the patient experienced 50 mL of blood loss, was administered 1300 mL of lactated Ringer’s solution, and excreted 500 mL of urine. After the operation, the double-lumen endotracheal tube was replaced with a single-lumen endotracheal tube and the patient was sent to the intensive care unit for monitoring and further treatment. Two hours after entering the intensive care unit, the patient was fully awake and the tracheal tube was smoothly removed. The following day, the patient was transferred to the thoracic surgery ward, where he recovered; he was discharged 12 days later. Postoperative chest computed tomography showed that the lungs were well inflated with no abnormalities ().