Central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection

Shouichi Ohga, Hidetoshi Takada, Keiko Honda, Takanori Inamura, Kenjiro Gondo, Kohichi Ohshima, Masahiro Yamamoto, Toshiro Hara

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Purpose: Central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. Patients and Methods: CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly and abnormal high titers of anti-Epstein-Barr virus (EBV) antibodies. At 8 years of age, he had a splenectomy because of progressive disease. Results: After 27 months of clinical remission, muscle weakness and paresthesia developed. Magnetic resonance imaging of his brain showed spotty T2 prolongation in left parietal, bilateral frontal, and temporal white matter with meningeal enhancement. Brain biopsy revealed the cerebral infiltration of CD3+, CD4+, CD8-, CD45RO+, CD56-, and EBV-encoded RNA 1+ cells. Conclusions: The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.

Original languageEnglish
Pages (from-to)42-46
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume21
Issue number1
DOIs
Publication statusPublished - Jan 1 1999

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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