systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
Findings
No curated finding names systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A 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.
Definition from the Mondo Disease Ontology (MONDO:0018223), read 2026-09-29. CC BY 4.0.
Where it sits
Other names
4 names
Resolves to: systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
- Also called
- systemic EBV-positive T-cell lymphoma of childhoodsystemic EBV-positive T-cell lymphoproliferative disease of childhoodsystemic EBV-positive T-cell lymphoproliferative disorder of childhoodsystemic EBV+ T-cell LPD of childhood