Persistent Polyclonal B-Cell Lymphocytosis (PPBL): An Entity That Is Not What it Seems

Authors

  • António Figueiredo Serviço de Patologia Clínica. Hospital Professor Doutor Fernando Fonseca. Amadora.
  • Ana Carolina Freitas Serviço de Hematologia. Instituto Português de Oncologia de Lisboa Francisco Gentil. Lisboa.
  • Diogo Paulino Serviço de Patologia Clínica. Instituto Português de Oncologia de Lisboa Francisco Gentil. Lisboa.
  • Carlos Severino Laboratório de Hematologia. Serviço de Patologia Clínica. Instituto Português de Oncologia de Lisboa Francisco Gentil. Lisboa.
  • Máriam Calú Laboratório de Hematologia. Serviço de Patologia Clínica. Instituto Português de Oncologia de Lisboa Francisco Gentil. Lisboa.
  • Rui Barreira Laboratório de Hematologia. Serviço de Patologia Clínica. Instituto Português de Oncologia de Lisboa Francisco Gentil. Lisboa.

DOI:

https://doi.org/10.20344/amp.19991

Keywords:

B-Lymphocytes, Lymphocytosis, Persistent Polyclonal B-Cell Lymphocytosis

Abstract

Persistent polyclonal B-cell lymphocytosis is a rare disease with chronic lymphocytosis of polyclonal origin, which is more frequent in mostly asymptomatic middle-aged female smokers. The hallmark of this entity is the presence of bilobed/binucleated B lymphocytes, which are polyclonal as demonstrated by immunophenotyping; an elevated IgM level is common. This disease shows, in most cases, an indolent course over many years and, although controversial, it may rarely convert to malignant lymphoma. In addition to smoking, a genetic predisposition for persistent polyclonal B-cell lymphocytosis is likely. Recurrent genetic aberrations have been described. The differential diagnosis includes non-Hodgkin’s lymphoma and a clear distinction between both entities is of the utmost importance because treatment is generally not indicated in the former: instead, regular follow-up is recommended. The authors describe the case of a 46-year-old female smoker, who presented with chronic lymphocytosis, elevated IgM and circulating binucleated lymphocytes. Excluding lymphoma was important considering the unusual presentation with constitutional symptoms and splenomegaly.

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References

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Published

2024-03-12

How to Cite

1.
Figueiredo A, Freitas AC, Paulino D, Severino C, Calú M, Barreira R. Persistent Polyclonal B-Cell Lymphocytosis (PPBL): An Entity That Is Not What it Seems. Acta Med Port [Internet]. 2024 Mar. 12 [cited 2024 Jul. 25];37(4):286-8. Available from: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/19991

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Section

Case Report