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Central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus
1Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Insights
Central nervous system (CNS) T cell lymphoproliferative disorder (T-LPD) is a rare complication of chronic active Epstein-Barr virus (CAEBV) infection. This case highlights CNS T-LPD in a young boy, emphasizing the need for vigilance in CAEBV patients.
Area of Science:
- Neurology
- Infectious Diseases
- Hematology
Background:
- Chronic active Epstein-Barr virus (CAEBV) infection can lead to serious complications.
- T cell lymphoproliferative disorders (T-LPD) are a group of aggressive lymphoid neoplasms.
Observation:
- A 14-month-old boy with CAEBV presented with fever, cytopenia, and hepatosplenomegaly.
- At 8 years old, he developed neurological symptoms including muscle weakness and paresthesia after a period of remission.
- Brain MRI revealed white matter changes and meningeal enhancement, with biopsy confirming cerebral infiltration by T cells and Epstein-Barr virus.
Findings:
- The patient was diagnosed with central nervous system (CNS) T cell lymphoproliferative disorder (T-LPD) associated with CAEBV.
- Cerebral infiltration was characterized by CD3+, CD4+, CD8-, CD45RO+, CD56-, and EBV-encoded RNA 1+ cells.
- The patient had no known underlying immunodeficiency.
Implications:
- CNS involvement in EBV-associated T-LPD is a rare but severe complication.
- Early recognition and diagnosis are crucial for managing T-LPD in CAEBV patients.
- This case underscores the importance of monitoring for neurological complications in individuals with CAEBV.
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.