A Boy with Chronic Active EBV Infection Presented as Mosquito Bite Hypersensitivity Progressed to Fatal Hemophagocytic Lymphohistiocytosis due to NK Cell Neoplasm

• Published in: Clinical pediatric hematology-oncology Vol. 26; no. 2; pp. 95 - 98
• Main Authors: Lee, Jin Ah, Han, Seung Beom, Chung, Nack Gyun, Kang, Jin Han, Kim, Myungshin, Jeong, Dae Chul
• Format: Journal Article
• Language: English
• Published: The Korean Society of Pediatric Hematology-Oncology 01.10.2019; 대한소아혈액종양학회
• Subjects: chronic active epstein-barr virus infection; hemophagocytic lymphohistiocytosis; mosquito bite hypersensitivity; nk cell neoplasm; 소아과학
• ISSN: 2233-5250; 2233-5250
• DOI: 10.15264/cpho.2019.26.2.95

Chronic active Epstein-Barr virus (CAEBV) infection is characterized by recurrent infectious mononucleosis (IM)-like symptoms and an unusual pattern of anti-EBV antibodies. We report a boy with CAEBV who progressed to aggressive hemophagocytic lymphohistiocytosis (HLH) with NK cell neoplasm. A 19-year-old adolescent boy was admitted with fever and a history of recurrent IM-like symptoms following mosquito bites since the age of 6 years. His condition was diagnosed as CAEBV with atypical lymphocytosis and an unusual pattern of anti-EBV antibodies. His symptoms subsided during treatment with steroids and cyclosporine, although the EBV genome load kept increasing for several years. He was re-admitted after follow-up loss for 8 years, and his clinical and laboratory findings confirmed HLH and high titer of the EBV genome. Bone marrow analysis with flow cytometry showed hemophagocytosis with compatible NK cell neoplasm. He rapidly progressed to pulmonary infection and expired soon after. We conclude that hematopoietic stem cell transplantation may be a potential therapeutic modality for treating CAEBV before serious EBV manifestations.