Persistent polyclonal B-cell lymphocytosis (PPBL)

  • Method:
  • Anticoagulant:
  • Recommendation:
  • Method:
    Cytomorphology
  • Anticoagulant:
    EDTA
  • Recommendation:
    obligatory
  • Method:
    Immunophenotyping
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    obligatory
  • Method:
    Chromosome analysis
  • Anticoagulant:
    Heparin
  • Recommendation:
    facultative
  • Method:
    FISH
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    facultative
  • Method:
    Molecular genetics
  • Anticoagulant:
  • Recommendation:
    no

Based on the current guidelines and the current state of research, there are different diagnostic recommendations for patients with persistent polyclonal B-cell lymphocytosis (PPBL). We have summarized the most important information on classification and diagnostic methods at MLL. In addition, we provide further literature on persistent polyclonal B-cell lymphocytosis so that you can inform yourself in more detail.

Persistent polyclonal B-cell lymphocytosis: Characteristics

Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare benign disease with chronic lymphocytosis of polyclonal origin, which occurs more frequently in middle-aged female smokers. These women are mostly asymptomatic with mild leukocytosis/lymphocytosis or show non-specific symptoms such as fatigue. Therefore, PPBL is often an incidental finding and no other underlying diseases are found, but an association with EBV infections has been discussed (Delage et al. 2001). Bilobulated, usually even binucleated B lymphocytes in the peripheral blood and a polyclonal increase of IgM in the serum are characteristics. Splenomegaly has been described in some cases (Cornet et al. 2009, Del Giudice et al. 2009). Lymphomas and secondary solid tumors appear to be more frequent in the year-long course, but this is controversial (Cornet et al. 2009, Cornet et al. 2016).

The actual cause of the disease persistent polyclonal B-cell lymphocytosis and the associated mechanism are unknown and probably represent a gradual process. In addition to the external factor "smoking", a genetic predisposition for persistent polyclonal B-cell lymphocytosis is very likely due to its gender-dependent and familial clustered occurrence (Carr et al. 1997, Delage et al. 2001).

Persistent polyclonal B-cell lymphocytosis: Diagnostic methods

Persistent polyclonal B-cell lymphocytosis: Prognosis

Persistent polyclonal B-cell lymphocytosis has several characteristics associated with malignant tumors. These include the occurrence of cytogenetic abnormalities and the frequently observed chromosomal instability (Mossafa et al. 2004, Cornet et al. 2016) as well as the detectability of various tumor-associated mutations (Stengel et al. 2018). Nevertheless, persistent polyclonal B-cell lymphocytosis often shows an indolent, stable course over many years or slight progress with continued cigarette consumption. Only in isolated cases of persistent polyclonal B-cell lymphocytosis has the occurrence of a malignant disease, e.g. lymphoma, been described and an association discussed (Del Giudice et al. 2009, Cornet et al. 2009, Cornet et al. 2016). The achievement of cytomorphological remission by stopping cigarette consumption is controversially discussed but observed (Mossafa et al. 1999, Cornet et al. 2009), the cytogenetic abnormalities seem to persist. A clear separation of malignant lymphomas is important, because due to the indolent course and the lack of malignancy, a therapy is generally not indicated. Regular follow-ups are recommended.

Status: June 2023

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