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Up to: Immune system cancer · Epstein-Barr virus-associated lymphoproliferative disorder · Mature T-cell and NK-cell non-Hodgkin lymphoma · Pediatric lymphoma · EBV-positive T-cell lymphoproliferative disorder of childhood
Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
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.
This condition has no sub-types.