A 47-year-old woman presented with intermittent cough, shortness of breath, fever, and body temperature up to 38.5 C, accompanied by right eyelid swelling around June 17, 2017, which worsened day by day. Weight loss was about 9 kg. On July 5, 2017, she visited a local hospital and received both eyes and head computed tomography (CT) scan, no obvious abnormalities were found. Chest CT showed pulmonary infection with a high possibility of fungal infection, and a small amount of pleural effusion in the left lung. She received antibiotics about 10 days, but the symptoms appreciated no improvement. Later, the patient had aggravated the above symptoms and developed high fever with the highest body temperature of 39.4 C. As a result, the patient was transferred to our hospital with "pulmonary infection" on July 17, 2017.
Physical examination included a high body temperature of 39 C, right eyelid swelling and a painless enlarged lymph node about 2 cm x 2 cm in size was in the left axilla. The auscultation of the lungs had coarse respiratory sounds with the moist rale. No hepatosplenomegaly and edema in lower limbs. Laboratory data revealed decreased leucocyte (1.50 x 10-9/L) and hemoglobin (88 g/L), raised aspartate transaminase (AST) (61 U/L), and lactate dehydrogenase (LDH) (724 U/L). Galactomannan (GM) test was positive and plasma (1,3)-beta-D-glucan (G) test was negative. Coagulation function, kidney function, ferritin, and erythrocyte sedimentation rate were all normal. An infectious origin was ruled out by sputum culture and acid-fast staining, and blood culture. HIV, hepatitis, and syphilis test panels were negative. Her autoimmune workup was negative. Reexamination of chest CT showed interstitial exudation in both lungs, mass lesions, and left pleural effusion, still considering fungal infection (Figure 1(a)). Bronchoscopy showed acute inflammatory changes in the bronchial mucosa. The above clinical findings indicated suspected aspergillosis pneumonia.
After symptomatic treatment with antibacterial, fungal, and leucocyte-elevating, her systemic symptoms such as fever, cough, and eyelid swelling improved, and leucocyte increased. Reexamination of orbital and chest CT showed no abnormalities in the orbit, but interstitial exudation of both lungs and left pleural effusion were more than the previous CT scan. On August 4, 2017, positron emission tomography (PET)-CT images showed multiple spherical lesions and ground glass-like increased images in the lungs, with significant lower lobe, and mostly hypermetabolic activity; left pleural and right interlobular effusion. Biopsy of hypermetabolic nodules in the chest wall revealed difficulty in differentiating inflammation and tumor, and PCR was negative for tuberculosis in paraffin-embedded tissue. Anti-infective treatment was continued and the patient was discharged 1 week later due to improvement in symptoms. However, fever returned after a brief respite and did not improve. She revisited our hospital on August 28, 2017. Reexamination of chest CT exhibited a reduced volume of multiple lesions of both lungs, and the interstitial exudation was slightly absorbed compared to before. A blood reexamination revealed re-decrease of leucocyte, higher levels of AST and LDH than in the first admission, and raised blood beta2 microglobulin (2549 ng/Ml). A of biopsy of the lung tissue was performed and revealed a high number of inflammatory cells infiltration in the pleural. Anti-infective treatment was ineffective, and her fever was unremitting, along with right thigh redness, swolling, heat, and painful (Figure 2(a) and (b)). On September 1, 2017, vascular ultrasound of the right lower limb showed intermuscular venous thrombosis, enlarged inguinal lymph nodes (multiple), and subcutaneous soft tissue edema with interstitial effusion on the lateral thigh. The patient underwent surgical decompression of fascial compartment and pathological biopsy of neoplasm (subcutaneous neoplasm, muscle interstitial neoplasm) of the right limb on September 4, 2017. Histopathological examination demonstrated focal rimming of individual adipocytes by infiltrating small to medium-sized atypical T lymphocytes with irregular and hyperchromatic nuclei. Immunohistochemistry exhibited positivity for CD3, CD8, granzyme B (GB), CD43, and T-cell intracellular antigen-1 (TIA-1), and negativity for CD4, CD56, CK, and CD79, Ki67 staining positive was 50% (Figure 3(a)-(d)), which led to a pathological diagnosis of SPTCL. A monoclonal rearrangement of TCR-beta gene was confirmed by polymerase chain reaction (PCR). Bone marrow aspiration suggested proliferative anemia and infection, and bone marrow biopsy had no lymphoma cells infiltration. A final diagnosis of SPTCL (IIB phase, IPI scores of 2) was made.
She was treated on September 15, 2017 with etoposide, vincristine, cyclophosphamide, doxorubicin, and prednisone (EPOCH) chemotherapy. After two cycles of chemotherapy, the patient's clinical symptoms were not significantly improved, she still had intermittent fever and shortness of breath, the enlarged lymph node in the left axillary did not noticeably shrunk, and the swelling of right thigh was not significantly reduced. Considering obvious systemic symptoms of the patients and chidamide is effective in PTCL, an attempt was made to add chidamide (30 mg BIW oral) to the chemotherapy from the third cycle. Fortunately, the patient responded well with resolution of all systemic manifestations of disease within four cycles of combined chemotherapy with chidamide, the patient had normal body temperature, significant improvement in cough and shortness of breath, and complete resolution of enlarged lymph node of left axillary and swelling of right thigh. Additionally, the chest CT showed that the lung lesions were significantly diminished (Figure 1(b)), and the right thigh magnetic resonance imaging (MRI) showed no obvious abnormal signal in subcutaneous and layers of muscle group (Figure 4). The efficacy was evaluated as CR, and patient was recommended to undergo HSCT as consolidation for long-term disease control, but the patient declined HSCT treatment, so she continued to underwent two cycles of EPOCH chemotherapy combined with chidamide. CR was evaluated again after six cycles of combined treatment, followed by chidamide monotherapy for maintenance. The last follow-up date was on January 16, 2020, and remission has been maintained. Written informed consent was obtained from the patient's legally authorized representative for publication of this case report and accompanying images.