Adult T cell lymphomas are neoplasms of CD4 positive T cells only observed in adults that have been infected by human T-lymphotropic virus type I (HTLV 1). The tumor cells contain clonal HTLV 1 provirus, which is thought to play a critical pathogenic role and the virus also encodes a protein called Tax that is a potent NF KB activator, which enhances lymphocyte growth and survival.
These cancers occur mainly in regions where HTLV-1 is endemic including southern Japan, West Africa, and the Caribbeans.
Most individuals with Adult T cell lymphoma present with skin lesions and may also have generalized lymphadenopathy, hepatosplenomegaly and hypercalcemia. Sometimes, the tumor may only involve the skin and can follow a much more indolent course.
Typically, this neoplasm has an aggressive course. Most patients present with rapidly progressive disease that can be fatal within months to a year of diagnosis despite aggressive chemotherapy.
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