An 18-year-old Saudi female who is single and with no medical background. On 11 October 2021, she presented to the emergency room with 3 weeks history of documented fever of up to 40 C associated with sweating and painful swelling on the right side of her neck. Her symptoms were associated with poor appetite, and she lost 5 kg. She denied any joint pain, skin rash, or oral ulcer. She had no bleeding tendency or exposure to chemotherapy or radiation. She visited her primary care physician, who prescribed an oral Co-amoxiclav without improvement over 5 days. She was vaccinated for COVID-19 in April 2021 and July 2021 with no complications.
She looked ill and febrile on examination, with a temperature of 38.7 C. Neck examination revealed multiple lymph nodes in the right anterior and posterior triangle varied in size of about 3-4 cm, freely mobile, mildly tender not attached to the deep structures. Otherwise, unremarkable chest, cardiovascular, and abdominal examination, with no palpable splenomegaly or hepatomegaly.
Her initial laboratory workup showed hemoglobin of 13.0 g/dL, a white blood cell (WBC) count was 2.65 x 103/microL, with neutropenia of 0.85 x 103/microL and lymphocyte of 1.60 x 103/microL, lactate dehydrogenase (LDH) of 550 U/L, ferritin 356 ng/mL, and erythrocyte sedimentation rate (ESR) was 19. C-reactive protein (CRP) was 2 mg/L (Table 1). Antinuclear antibody (ANA) was1:80 (homogeneous pattern), and the QuantiFERON test for tuberculosis was negative. Full virology screening, including coronavirus disease 2019 (COVID-19), hepatitis B virus (HBV), hepatitis C virus (HCV), human immunodeficiency virus (HIV), cytomegalovirus (CMV), and Epstein-Barr virus (EBV) were non-reactive. Peripheral blood film showed leucopenia and neutropenia, and was negative for blast cells. The analysis of triglyceride and interleukin-2 receptor (IL-2R) was not done due to unavailability in our center at that time.
The patient was admitted to the medical ward from the 11th of October 2021 until the 27th of October for a workup; she continued to have fever spikes and poor oral intake. On the second day of admission, bone marrow and lymph node biopsies were performed. After that, she developed an erythematous maculopapular rash in the extensor surfaces of her lower limbs, which was thought to be a reaction related to the perioperative prophylactic Cefuroxime given at the time of the excisional lymph node biopsy. The skin eruption recurred 3 days later, and a punch skin biopsy showed minimal superficial dermal perivascular lymphocytic cell infiltrate consistent with perivascular interface dermatitis, a possible morbilliform drug eruption (Figure 1). Computer tomography of the neck revealed bilateral sub-centimeter cervical lymph nodes; some lymph nodes showed central necrosis (Figure 2). Computer tomography of the abdomen and pelvis showed multiple mildly prominent para-aortic lymph nodes are seen larger one measuring 13 mm x 7 mm, and a few bilateral external iliac nodes are seen, a larger one at 11 mm x 5 mm. Solid abdominal viscera appear grossly unremarkable. Moreover, lymph node histopathology showed partially effaced lymph nodes composed of histiocytes, lymphocytes, scattered eosinophils, karyorrhexis, and apoptotic bodies in a necrotic background with immunohistochemistry diffuse and strongly positive for CD68 (Figure 3).
Bone marrow morphology revealed the presence of hemophagocytes and no blast cells. Active and normal production of the three cell lineages is seen. Limited flow cytometry showed 14% lymphocytes with 70% T cells (positive CD2, CD3, CD5, CD7, CD8), 25% B cells (CD19), and NK-cell of 5% (CD5-/CD19-) (Figure 4).
Based on investigation results and pathological findings, a multidisciplinary meeting was done, and the patient was started on oral dexamethasone 20 mg P.O. daily for 5 days. The patient dramatically improved, and the fever subsided completely. She regained her appetite; the skin rash did not recur, the size of the cervical lymphadenopathy regressed, and white cell counts rose to near normal.