T-Cell Prolymphocytic Leukemia Involving Lymph Nodes and Other Tissues
T-cell prolymphocytic leukemia (T-PLL) is an aggressive T-cell leukemia composed of small- to medium-sized prolymphocytes with a mature T-cell immunophenotype. T-PLL represents ~2 % of mature lymphocytic leukemias. This neoplasm affects mostly adults, with a median age of 65 years (range, 30–94 years) and a male to female ratio of 2:1. Patients with ataxia telangiectasia are at increased risk of developing T-PLL. Most patients present with B-symptoms, hepatosplenomegaly, and generalized lymphadenopathy. Patients commonly have marked leukocytosis and the lymphocyte count usually exceeds 100 × 109/L. Anemia and thrombocytopenia also occur, but less commonly. Serologic testing for human T-cell leukemia virus type 1 (HTLV-1) is negative.
KeywordsAtaxia Telangiectasia High Endothelial Venule Generalize Lymphadenopathy Prolymphocytic Leukemia Mark Leukocytosis
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