Clin Pediatr Hematol Oncol.  2022 Oct;29(2):79-83. 10.15264/cpho.2022.29.2.79.

Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Resembling Recurrent Follicular Lymphoma: A Case Report

• Park CH
• Choi YB

• KMID: 2534768
• DOI: http://doi.org/10.15264/cpho.2022.29.2.79

Abstract

Epstein-Barr virus (EBV)-associated lymphoproliferative diseases represent various clinical manifestations. EBV-associated hemophagocytic lymphohistiocytosis (HLH) and lymphoma are difficult to distinguish due to overlapping characteristics between these two diseases. Here, we present a case of EBV-associated HLH, mimicking disease relapse. A 13-year-old boy with follicular lymphoma presented with fever and dyspnea. The patient was diagnosed with follicular lymphoma (FL) two months prior to the visit and underwent two cycles of CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. Physical examination revealed multiple enlarged cervical lymph nodes and hepatomegaly while the following findings were obtained by laboratory data: white blood cell count, 200 cells/L; hemoglobin concentration, 9.9 g/dL; platelet count, 55,000 platelets/L; and ferritin level, 3,290 µg/L. Positron-emission tomography revealed newly developed lesions in the neck, chest, and abdominal lymph nodes with fluorine-18 fluoro-deoxy-glucose uptakes not visible at the time of diagnosis. Disease recurrence was suspected by imaging, and the patient underwent a neck lymph node biopsy, following which the chemotherapy regimen was changed. The neck lymph node biopsy revealed hemophagocytosis, and EB-encoding region in situ hybridization revealed scattered cells which were positive for CD3 and CD8 but negative for CD20 and CD56. Finally, the patient was diagnosed with EBV-associated HLH but not with recurrent FL. Therefore, a biopsy is essential for the final diagnosis.

Keyword

Follicular lymphoma; Epstein-Barr virus; Hemophagocytic lymphohistiocytosis