Persistent Polyclonal B-Cell Lymphocytosis (PPBL): An Entity That Is Not What it Seems
DOI:
https://doi.org/10.20344/amp.19991Keywords:
B-Lymphocytes, Lymphocytosis, Persistent Polyclonal B-Cell LymphocytosisAbstract
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.
Downloads
References
Gordon DS, Jones BM, Browning SW, Spira TJ, Lawrence DN. Persistent polyclonal lymphocytosis of B lymphocytes. N Engl J Med. 1982;307:232-6. DOI: https://doi.org/10.1056/NEJM198207223070407
Online Mendelian Inheritance in Man, OMIM®. Johns Hopkins University, Baltimore, MD. MIM 606445: Date last edited 7/09/2015. [consultado 2023 abr 04]. Disponível em: https://omim.org/.
Cornet E, Lesesve JF, Mossafa H, Sébahoun G, Levy V, Davi F, et al. Long-term follow-up of 111 patients with persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes. Leukemia. 2009;23:419-22. DOI: https://doi.org/10.1038/leu.2008.208
Troussard X, Flandrin G. Chronic B-cell lymphocytosis with binucleated lymphocytes (LWBL): a review of 38 cases. Leuk Lymphoma. 1996;20:275-9. DOI: https://doi.org/10.3109/10428199609051618
Troussard X, Cornet E, Lesesve JF, Kourel C, Mossafa H. Polyclonal B-cell lymphocytosis with binucleated lymphocytes (PPBL). Onco Targets Ther. 2008;1:59-66. DOI: https://doi.org/10.2147/OTT.S4182
Delage R, Jacques L, Massinga-Loembe M, Poulin J, Bilodeau D, Mignault C, et al. Persistent polyclonal B-cell lymphocytosis: further evidence for a genetic disorder associated with B-cell abnormalities. Br J Haematol. 2001;114:666-70. DOI: https://doi.org/10.1046/j.1365-2141.2001.02975.x
Feugier P, De March AK, Lesesve JF, Monhoven N, Dorvaux V, Braun F, et al. Intravascular bone marrow accumulation in persistent polyclonal lymphocytosis: a misleading feature for B-cell neoplasm. Mod Pathol. 2004;17:1087-96. DOI: https://doi.org/10.1038/modpathol.3800156
Mossafa H, Tapia S, Flandrin G, Troussard X. Chromosomal instability and ATR amplification gene in patients with persistent and polyclonal B-cell lymphocytosis (PPBL). Leuk Lymphoma. 2004;45:1401-6. DOI: https://doi.org/10.1080/10428194042000191738
Delage R, Roy J, Jacques L, Bernier V, Delage JM, Darveau A. Multiple bcl-2/ Ig gene rearrangements in persistent polyclonal B-cell lymphocytosis. Br J Haematol. 1997;97:589-95. DOI: https://doi.org/10.1046/j.1365-2141.1997.852725.x
Sun P, Juskevicius R. Histological and immunohistochemical features of the spleen in persistent polyclonal B- cell lymphocytosis closely mimic splenic B-cell lymphoma. Diagn Pathol. 2012;7:107. DOI: https://doi.org/10.1186/1746-1596-7-107
Del Giudice I, Pileri SA, Rossi M, Sabattini E, Campidelli C, Starza ID, et al. Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly. Br J Haematol. 2009;144:726-31. DOI: https://doi.org/10.1111/j.1365-2141.2008.07551.x
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2024 Acta Médica Portuguesa
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
All the articles published in the AMP are open access and comply with the requirements of funding agencies or academic institutions. The AMP is governed by the terms of the Creative Commons ‘Attribution – Non-Commercial Use - (CC-BY-NC)’ license, regarding the use by third parties.
It is the author’s responsibility to obtain approval for the reproduction of figures, tables, etc. from other publications.
Upon acceptance of an article for publication, the authors will be asked to complete the ICMJE “Copyright Liability and Copyright Sharing Statement “(http://www.actamedicaportuguesa.com/info/AMP-NormasPublicacao.pdf) and the “Declaration of Potential Conflicts of Interest” (http:// www.icmje.org/conflicts-of-interest). An e-mail will be sent to the corresponding author to acknowledge receipt of the manuscript.
After publication, the authors are authorised to make their articles available in repositories of their institutions of origin, as long as they always mention where they were published and according to the Creative Commons license.
