/systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood

systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood

Rare Disease

MONDO:0018223

Also Known As

EBV-positive T-cell lymphoproliferative disorder of childhoodsystemic EBV+ T-cell LPD of childhoodsystemic EBV-positive T-cell lymphoma of childhoodsystemic EBV-positive T-cell lymphoproliferative disease of childhoodsystemic EBV-positive T-cell lymphoproliferative disorder of childhood

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.