mature T-cell and NK-cell non-Hodgkin lymphoma immune system cancer pediatric lymphoma EBV-positive T-cell lymphoproliferative disorder of childhood Epstein-Barr virus-associated lymphoproliferative disorder systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood https://www.malacards.org/card/systemic_epstein_barr_virus_positive_t_cell_lymphoma_of_childhood_2 https://www.malacards.org/card/systemic_epstein_barr_virus_positive_t_cell_lymphoproliferative_disease_of_childhood systemic EBV-positive T-cell lymphoma of childhood MONDO:0018223 SCTID:721311006 systemic EBV-positive T-cell lymphoproliferative disorder of childhood UMLS:C2699747 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. systemic EBV+ T-cell LPD of childhood Orphanet:364033 DOID:0070324 EBV-positive T-cell lymphoproliferative disorder of childhood ICDO:9724/3 NCIT:C80374 systemic EBV-positive T-cell lymphoproliferative disease of childhood GARD:0021562 MEDGEN:397492