Central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection.
OBJECTIVE
Central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported.


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.
Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
S
S
Ohga
H
H
Takada
K
K
Honda
T
T
Inamura
K
K
Gondo
K
K
Ohshima
M
M
Yamamoto
T
T
Hara
D000208:Acute Disease; D000914:Antibodies, Viral / Q000097:blood; D001927:Brain Diseases / Q000473:pathology* / Q000821:virology*; D002648:Child; D002908:Chronic Disease; D018450:Disease Progression; D006566:Herpesviridae Infections / Q000150:complications* / Q000473:pathology / Q000821:virology; D004854:Herpesvirus 4, Human; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000473:pathology* / Q000601:surgery / Q000821:virology*; D008297:Male; D013156:Splenectomy; D013601:T-Lymphocytes; D014412:Tumor Virus Infections / Q000150:complications* / Q000473:pathology / Q000821:virology
D000914:Antibodies, Viral