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Prolymphocytic leukemia.

R M Stone1

  • 1Department of Medicine, Dana-Farber Cancer Institute, Boston, Massachusetts.

Hematology/Oncology Clinics of North America
|April 1, 1990
PubMed
Summary
This summary is machine-generated.

Prolymphocytic leukemia (PLL) is an aggressive B-cell malignancy distinct from chronic lymphocytic leukemia (CLL). Combination chemotherapy shows promise for treating this rare but severe lymphoid disorder.

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Prolymphocytic leukemia (PLL) is a rare variant of chronic lymphocytic leukemia (CLL).
  • PLL involves malignant transformation of a B lymphocyte at an intermediate developmental stage.
  • PLL cells exhibit distinct immunophenotypes, including heavy surface IgM/IgD staining and loss of mouse red blood cell receptors.

Purpose of the Study:

  • To describe the clinical and morphologic characteristics of PLL.
  • To differentiate PLL from typical CLL based on cellular origin and presentation.
  • To evaluate potential treatment strategies for PLL.

Main Methods:

  • Immunophenotypic analysis of PLL cells.
  • Morphologic examination of prolymphocytes.

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  • Cytogenetic analysis, focusing on chromosome 14 abnormalities.
  • Review of clinical course and treatment outcomes.

    • Main Results:

    • PLL presents with massive splenomegaly and extremely high white blood cell counts (>100,000/mm3) but minimal lymphadenopathy.
    • Prolymphocytes are large cells with open chromatin and prominent nucleoli.
    • Extra material on chromosome 14q is the most common cytogenetic abnormality.
    • PLL follows an aggressive clinical course with poor median survival.

    Conclusions:

    • PLL is a distinct entity from CLL, originating from a more mature B cell.
    • Aggressive combination chemotherapy, similar to that used for non-Hodgkin's lymphomas, may be more effective than CLL-specific oral regimens for treating PLL.