Clinical course and features of persistent polyclonal B-cell lymphocytosis with BCL-6 amplification during pregnancy

Eur Rev Med Pharmacol Sci. 2023 Apr;27(8):3514-3518. doi: 10.26355/eurrev_202304_32124.

Abstract

Background: Persistent polyclonal B-cell lymphocytosis is a rare nonmalignant disorder characterized by mild persistent lymphocyte proliferation with possible evolution to aggressive lymphoma. Its biology is not well known, but it is characterized by a specific immunophenotype with rearrangement of the BCL-2/IGH gene, whereas amplification of the BCL-6 gene has rarely been reported. Given the paucity of reports, it has been hypothesized that this disorder is associated with poor pregnancy outcomes.

Case report: To our knowledge, only two successful pregnancies have been described in women with this condition. We report the third successful pregnancy in a patient with PPBL and the first with amplification of the BCL-6 gene.

Conclusions: PPBL is still a poorly understood clinical condition with insufficient data to demonstrate an adverse effect on pregnancy. The role of BCL-6 dysregulation in the pathogenesis of PPBL and its prognostic significance are still unknown. Evolution into aggressive clonal lymphoproliferative disorders is possible and prolonged hematologic follow-up is warranted in patients with this rare clinical disorder.

Publication types

  • Case Reports

MeSH terms

  • B-Lymphocytes* / pathology
  • Disease Progression
  • Female
  • Genes, bcl-2
  • Humans
  • Lymphocytosis* / diagnosis
  • Lymphocytosis* / genetics
  • Pregnancy

Substances

  • BCL6 protein, human

Supplementary concepts

  • Persistent Polyclonal B-Cell Lymphocytosis