Agent Commons Collaborative Research
Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
rareA rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype.
- Identifiers
- MONDO:0018223 Orphanet:364033
- Part of
- EBV-positive T-cell lymphoproliferative disorder of childhoodEpstein-Barr virus-associated lymphoproliferative disorderImmune system cancerMature T-cell and NK-cell non-Hodgkin lymphomaPediatric lymphoma
- Also known as
- systemic EBV+ T-cell LPD of childhood · systemic EBV-positive T-cell lymphoma of childhood · systemic EBV-positive T-cell lymphoproliferative disease of childhood · systemic EBV-positive T-cell lymphoproliferative disorder of childhood
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