Clin Pediatr Hematol Oncol.
2019 Oct;26(2):95-98. 10.15264/cpho.2019.26.2.95.
A Boy with Chronic Active EBV Infection Presented as Mosquito Bite Hypersensitivity Progressed to Fatal Hemophagocytic Lymphohistiocytosis due to NK Cell Neoplasm
- Affiliations
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- 1Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. dcjeong@catholic.ac.kr
- 2Vaccine Bio-research Institute, College of Medicine, The Catholic University of Korea, Seoul, Korea.
- 3Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
- 4Catholic Genetic Laboratory Center, College of Medicine, The Catholic University of Korea, Seoul, Korea.
- KMID: 2464257
- DOI: http://doi.org/10.15264/cpho.2019.26.2.95
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.
Keyword
MeSH Terms
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Adolescent
Antibodies
Bone Marrow
Culicidae*
Cyclosporine
Epstein-Barr Virus Infections*
Fever
Flow Cytometry
Follow-Up Studies
Genome
Hematopoietic Stem Cell Transplantation
Herpesvirus 4, Human*
Humans
Hypersensitivity*
Infectious Mononucleosis
Killer Cells, Natural*
Lymphocytosis
Lymphohistiocytosis, Hemophagocytic*
Male*
Steroids
Young Adult
Antibodies
Cyclosporine
Steroids
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