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

MONDO:0018223

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.

Also known as: EBV-positive T-cell lymphoproliferative disorder of childhood, 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

3266 clinical trials for this condition and its sub-types, 2 tagged with Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood itself.

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