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Case Report
A Boy with Chronic Active EBV Infection Presented as Mosquito Bite Hypersensitivity Progressed to Fatal Hemophagocytic Lymphohistiocytosis due to NK Cell Neoplasm
Clin Pediatr Hematol Oncol 2019;26:95-8.
Published online October 31, 2019
© 2019 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Jin Ah Lee1, Seung Beom Han1,2, Nack Gyun Chung1, Jin Han Kang1,2, Myungshin Kim3,4 and Dae Chul Jeong1,2

1Department of Pediatrics, 2Vaccine Bio-research Institute, 3Department of Laboratory Medicine, 4Catholic Genetic Laboratory Center, College of Medicine, The Catholic University of Korea, Seoul, Korea
Correspondence to: Dae Chul Jeong
Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 06591, Korea
Tel: +82-2-2258-6180
Fax: +82-2-537-4544
E-mail: dcjeong@catholic.ac.kr
ORCID ID: orcid.org/0000-0003-0934-817X
Received September 10, 2019; Revised October 7, 2019; Accepted October 17, 2019.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
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.
Keywords: Chronic active Epstein-Barr virus infection, Hemophagocytic lymphohistiocytosis, NK cell neoplasm, Mosquito bite hypersensitivity
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October 2019, 26 (2)
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  • Dae Chul Jeong