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This neoplasm of childhood is characterized by a clonal proliferation of EBV-infected T-cells with an activated cytotoxic phenotype. It can occur shortly after primary acute EBV infection or in the setting of chronic active EBV infection (CAEBV).
This entity shows some overlapping clinicopathologic features with aggressive NK-cell leukemia.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9702/3).
Systemic EBV-positive T-cell lymphoproliferative disease of childhood is one of two major types of Epstein-Barr (EBV) associated T-cell lymphoproliferative disorders that have been reported in the pediatric age group. (See also 9725/3: Hydroa vacciniforme-like lymphoma)
Systemic EBV-positive T-cell lymphoproliferative disease of childhood is a systemic disease of children and young adults. The etiology of this neoplasm is not known. A genetic defect in the immune response to EBV is suspected.
Most cases have a fulminant clinical course with multiple organ failure and sepsis, resulting in death, usually within days or weeks of diagnosis. Some cases have a subacute course of several months to a year.