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Related Experiment Videos

Large cell lymphoma complicating persistent polyclonal B cell lymphocytosis

J Roy1, C Ryckman, V Bernier

  • 1Division of Hematology, Saint Sacrement Hospital, Quebec City, Canada.

Leukemia
|July 17, 1998
PubMed
Summary

Persistent polyclonal B cell lymphocytosis (PPBL) can rarely progress to non-Hodgkin's lymphoma. This case highlights the potential, though rare, for B cell malignancy in patients with PPBL.

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Persistent polyclonal B cell lymphocytosis (PPBL) is a rare disorder with an unknown long-term prognosis.
  • The potential for PPBL to transform into B cell malignancy is not well-defined.

Observation:

  • A 39-year-old female with a 19-year history of PPBL was diagnosed with stage IV-B large cell lymphoma.
  • The patient's lymphoma was rapidly fatal despite aggressive treatment including chemotherapy and stem cell transplantation.
  • bcl-2/Ig gene rearrangements were detected in peripheral blood mononuclear cells but absent in lymphoma cells.
  • Epstein-Barr virus (EBV) was not detected in lymphoma cells despite serological evidence of past exposure.

Findings:

  • Non-Hodgkin's lymphoma can occur in the context of persistent polyclonal B cell lymphocytosis.

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  • The bcl-2/Ig gene rearrangement, found in PPBL, was not present in the lymphoma cells of this patient.
  • Epstein-Barr virus (EBV) did not appear to be directly involved in the pathogenesis of the lymphoma in this case.

    • Implications:

    • This case suggests a potential, albeit rare, risk of hematologic malignancy transformation in patients with PPBL.
    • Further long-term studies with larger patient cohorts are necessary to accurately assess the risk of malignancy in PPBL.
    • Understanding the natural history of PPBL is crucial for early detection and management of potential complications like lymphoma.