Persistent Polyclonal B-Cell Lymphocytosis (PPBL) : An Entity That Is Not What it Seems
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:37 |
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| Enthalten in: |
Acta medica portuguesa - 37(2024), 4 vom: 01. Apr., Seite 286-288 |
| Sprache: |
Portugiesisch |
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| Weiterer Titel: |
Linfocitose Policlonal Persistente de Células B (LPPB): Uma Entidade que Não É o que Parece |
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| Beteiligte Personen: |
Figueiredo, António [VerfasserIn] |
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| Links: |
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| Themen: |
B-Lymphocytes |
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| Anmerkungen: |
Date Completed 22.04.2024 Date Revised 22.04.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.20344/amp.19991 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM369606825 |
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| 246 | 3 | 3 | |a Linfocitose Policlonal Persistente de Células B (LPPB): Uma Entidade que Não É o que Parece |
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| 520 | |a 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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