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Polyclonal B-cell lymphocytosis with binucleated lymphocytes (PPBL).

Xavier Troussard1, Edouard Cornet, Jean-François Lesesve

  • 1Laboratoire d'Hématologie Côte de Nacre, Université Caen Basse Normandie Caen, Registre Régional des Hémopathies Malignes de Basse Normandie, France.

Oncotargets and Therapy
|December 4, 2010
PubMed
Summary

Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare condition diagnosed in women, often smokers, characterized by chronic lymphocytosis and a specific chromosomal abnormality. Long-term follow-up is crucial as PPBL may evolve into clonal proliferation or malignancy.

Keywords:
+i(3q)binucleated lymphocytespolyclonal lymphocytosispremature chromosome condensation

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Published on: October 23, 2019

Area of Science:

  • Hematology
  • Oncology
  • Cytogenetics

Background:

  • Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare hematologic disorder.
  • Typically affects women, often smokers, presenting with chronic absolute lymphocytosis.
  • Distinguished from B-cell chronic lymphoproliferative disorders by polyclonal B cells and specific genetic markers.

Purpose of the Study:

  • To analyze the diagnostic markers and long-term clinical course of PPBL.
  • To investigate the frequency of the specific chromosomal abnormality +i(3q) in PPBL.
  • To assess the risk of malignant transformation or other serious complications in PPBL patients.

Main Methods:

  • Conventional cytogenetic analysis (CCA) and fluorescence in situ hybridization (FISH) were performed on 111 PPBL patients.
  • Patients were followed for a median of 4.4 years.
  • Clinical and biological data, including development of malignancies and monoclonal gammopathies, were recorded.

Main Results:

  • The extra isochromosome for the long arm of chromosome 3 (+i(3q)) was detected in 34% of cases by CCA and 71% by FISH.
  • Combining both methods identified +i(3q) in a higher percentage of patients.
  • Most patients had a stable course, but 6 died from various causes, and 3 developed non-Hodgkin's lymphoma (NHL).

Conclusions:

  • PPBL is associated with the specific chromosomal abnormality +i(3q), detectable by both CCA and FISH.
  • While often stable, PPBL carries a risk of evolving into clonal proliferation, NHL, or secondary solid cancers.
  • Long-term clinical and biological follow-up is essential for all PPBL patients.