SEER is an authoritative source of information on cancer incidence and survival in the United States. SEER currently collects and publishes cancer incidence and survival data from population-based cancer registries covering approximately 28 percent of the U.S. population.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9702/3).
Systemic EBV-positive T-celllymphoproliferative disease of childhood is one of two major types of Epstein-Barr (EBV) associated T-celllymphoproliferative disorders that have been reported in the pediatric age group. (See also 9725/3: Hydroa vacciniforme-like lymphoma)
Systemic EBV-positive T-celllymphoproliferative 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.