T-cell Prolymphocytic Leukemia

T-cell Prolymphocytic Leukemia

Kathryn Foucar, MD

This peripheral blood smear shows a striking leukocytosis, which exceeded 530 × 109/L in this elderly woman. Hemoglobin was preserved while platelets were mildly decreased in this case of T-cell prolymphocytic leukemia.

The leukocytes in this case of T-prolymphocytic leukemia show very distinctive nuclear and cytoplasmic features. Note the nuclear irregularity and cytoplasmic blebbing.



  • T-cell prolymphocytic leukemia (T-PLL)


  • T-cell chronic lymphocytic leukemia


  • Mature T-cell leukemia composed of small to medium-sized lymphocytes

    • Variably prominent nucleoli

    • Variable nuclear irregularity

    • Scant to moderate amounts of cytoplasm

  • Recurrent cytogenetic finding: inv(14)(q11q32)



  • Key leukemogenic role of TCL1 oncoprotein overexpression

  • Translocations of TCL1 gene(s) into promoter/enhancer regions of TCRα/δ dysregulates TCL1

  • ATM deletions and haploinsufficiency of CDKN1B also leukemogenic



  • Incidence

    • 2% of chronic leukemias in adults

    • Increased incidence in patients with ataxia telangiectasia

    • No association with environmental exposure

    • No link to infections established

  • Age

    • Median: 65 years (range: 30-90 years)

  • Gender

    • Male predominance (3:1)

  • Ethnicity

    • No key ethnic associations


  • Blood, bone marrow, spleen, liver, lymph nodes

  • Less often skin, effusions


  • 90% of patients symptomatic

  • Key symptoms include abdominal distension, bulky lymph nodes, skin rash

  • Marked splenomegaly and hepatomegaly typical

  • Skin lesions and pleural effusions in subset

Laboratory Tests

  • Key laboratory tests include CBC with differential, flow cytometric immunophenotyping, and conventional cytogenetics

  • CBC shows marked lymphocytosis (WBC > 100 × 109/L in 50% of cases)

  • Additional CBC findings include anemia (25%) and thrombocytopenia (50%)

Natural History

  • Aggressive disease; 20% overall 5-year survival rate

  • Better outcome for responders to CAMPATH-1H


  • Drugs

    • Various agents used, including purine analogs

    • Best results achieved with humanized monoclonal antibody alemtuzumab (anti-CD52) (CAMPATH-1H)

    • Newer treatments include

      • Other monoclonal antibody agents

      • Nonmyeloablative stem cell transplant

      • Radioimmunotherapy


  • Poor; median survival of non-CAMPATH-1H responders is 4 months

  • Overall median survival is about 2 years

  • 20% 5-year survival

Jun 13, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on T-cell Prolymphocytic Leukemia
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