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T-Cell Prolymphocytic Leukemia Involving Lymph Nodes and Other Tissues

  • Roberto N. Miranda
  • Joseph D. Khoury
  • L. Jeffrey Medeiros
Chapter
Part of the Atlas of Anatomic Pathology book series (AAP)

Abstract

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.

Keywords

Ataxia Telangiectasia High Endothelial Venule Generalize Lymphadenopathy Prolymphocytic Leukemia Mark Leukocytosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Roberto N. Miranda
    • 1
  • Joseph D. Khoury
    • 1
  • L. Jeffrey Medeiros
    • 1
  1. 1.Department of HematopathologyThe University of Texas M.D. Anderson Cancer CenterHoustonUSA

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