Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease 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.

Also known as 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, systemic EBV+ T-cell LPD of childhood — per MONDO

Also identified as

• DOID 0070324 per MONDO
• NCIT C80374 per MONDO
• Orphanet 364033 per MONDO
• SCTID 721311006 per MONDO
• UMLS C2699747 per MONDO

Affected Organs