T-Cell Prolymphocytic Leukemia Involving Lymph Nodes and Other Tissues

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


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.


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.


  1. 1.
    Muzzafar T, Medeiros LJ. T-cell prolymphocytic leukemia involving lymph node and other tissues. In: Medeiros LJ, editor. Diagnostic pathology, lymph nodes and spleen with extranodal lymphomas. 1st ed. Altona: Amirsys; 2011. p. 10-72–9.Google Scholar
  2. 2.
    Catovsky D, Muller-Hermelink HK, Ralfkiaer E. T-cell prolymphocytic leukaemia. In: Swerdlow SH, Harris NL, Jaffe ES, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 270–1.Google Scholar
  3. 3.
    Garand R, Goasguen J, Brizard A, et al. Indolent course as a relatively frequent presentation in T-prolymphocytic leukaemia. Groupe Francais d’Hematologie Cellulaire. Br J Haematol. 1998;103:488–94.PubMedCrossRefGoogle Scholar
  4. 4.
    Mallett RB, Matutes E, Catovsky D, et al. Cutaneous infiltration in T-cell prolymphocytic leukaemia. Br J Dermatol. 1995;132:263–6.PubMedCrossRefGoogle Scholar
  5. 5.
    Osuji N, Matutes E, Catovsky D, et al. Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review. Am J Surg Pathol. 2005;29:935–41.PubMedCrossRefGoogle Scholar
  6. 6.
    Herling M, Khoury JD, Washington LT, et al. A systematic approach to diagnosis of mature T-cell leukemias reveals heterogeneity among WHO categories. Blood. 2004;104(2):328–35.Google Scholar
  7. 7.
    Dearden CE, Matutes E, Cazin B, et al. High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H. Blood. 2001;98:1721–6.PubMedCrossRefGoogle Scholar
  8. 8.
    Dearden CE, Khot A, Else M, et al. Alemtuzumab therapy in T-cell prolymphocytic leukemia: comparing efficacy in a series treated intravenously and a study piloting the subcutaneous route. Blood. 2011;118:5799–802.PubMedCrossRefGoogle Scholar
  9. 9.
    Brito-Babapulle V, Hamoudi R, Matutes E, et al. p53 allele deletion and protein accumulation occurs in the absence of p53 gene mutation in T-prolymphocytic leukaemia and Sezary syndrome. Br J Haematol. 2000;110:180–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Brito-Babapulle V, Catovsky D. Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia. Cancer Genet Cytogenet. 1991;55:1–9.PubMedCrossRefGoogle Scholar
  11. 11.
    Costa D, Queralt R, Aymerich M, et al. High levels of chromosomal imbalances in typical and small-cell variants of T-cell prolymphocytic leukemia. Cancer Genet Cytogenet. 2003;147:36–43.PubMedCrossRefGoogle Scholar

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

Personalised recommendations