Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
Synonyms: Systemic EBV+ T-cell LPD of childhood | Systemic EBV-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.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version September 2023.
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Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood?
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