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

Central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly, and abnormal high titers of anti-Epstein-Barr...

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Veröffentlicht in:Journal of pediatric hematology/oncology 1999, Vol.21 (1), p.42-46
Hauptverfasser: OHGA, S, TAKADA, H, HONDA, K, INAMURA, T, GONDO, K, OHSHIMA, K, YAMAMOTO, M, HARA, T
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container_title Journal of pediatric hematology/oncology
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creator OHGA, S
TAKADA, H
HONDA, K
INAMURA, T
GONDO, K
OHSHIMA, K
YAMAMOTO, M
HARA, T
description Central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. 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. 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. The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.
doi_str_mv 10.1097/00043426-199901000-00009
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Malignant lymphomas. Malignant reticulosis. 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subjects Acute Disease
AIDS/HIV
Antibodies, Viral - blood
Biological and medical sciences
Brain Diseases - pathology
Brain Diseases - virology
Child
Chronic Disease
Disease Progression
Hematologic and hematopoietic diseases
Herpesviridae Infections - complications
Herpesviridae Infections - pathology
Herpesviridae Infections - virology
Herpesvirus 4, Human
Humans
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymphoproliferative Disorders - pathology
Lymphoproliferative Disorders - surgery
Lymphoproliferative Disorders - virology
Male
Medical sciences
Splenectomy
T-Lymphocytes
Tumor Virus Infections - complications
Tumor Virus Infections - pathology
Tumor Virus Infections - virology
title Central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection
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