systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
http://purl.obolibrary.org/obo/MONDO_0018223
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is 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. [ Orphanet:364033 ]
Term info
- UMLS:CN204753 (MONDO:equivalentTo)
- SCTID:721311006 (MONDO:equivalentTo)
- Orphanet:364033 (MONDO:equivalentTo)
- DOID:0070324 (MONDO:equivalentTo)
- NCIT:C80374 (MONDO:equivalentTo)
- ICDO:9724/3 (NCIT:C80374)
ordo_disease
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is 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.
http://purl.obolibrary.org/obo/DOID_0070324, http://identifiers.org/snomedct/721311006, http://purl.obolibrary.org/obo/Orphanet_364033, http://linkedlifedata.com/resource/umls/id/CN204753, http://purl.obolibrary.org/obo/NCIT_C80374
EBV-positive T-cell lymphoproliferative disorder of childhood
systemic EBV-positive T-cell lymphoma of childhood, systemic EBV+ T-cell LPD of childhood, systemic EBV-positive T-cell lymphoproliferative disease of childhood, systemic EBV-positive T-cell lymphoproliferative disorder of childhood
MONDO:0018223