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

Here you can inform yourself about characteristics, diagnostics and prognosis of persistent polyclonal B-cell lymphocytosis.

Characteristics

Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare benign disease with chronic lymphocytosis of polyclonal origin, first described in 1982, which occurs more frequently in middle-aged female smokers. These are mostly mild lymphocytosis and are mostly symptom-free or show unspecific symptoms such as fatigue. Often, therefore, the findings are random and no other underlying diseases are found, but an association with EBV infections has been discussed (Delage et al. 2001). Typical for the disease are bilobulated, mostly even binuclear B-lymphocytes in peripheral blood as well as a polyclonal increase of IgM in serum. In some cases splenomegaly has been described (Cornet et al. 2009, Del Giudice et al. 2009). Lymphomas and secondary solid tumours seem to occur more frequently over the years, but this is still controversially discussed (Cornet et al. 2009, Cornet et al. 2016).

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

Persistent polyclonal B-cell lymphocytosis: Diagnostics

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.

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