Atlas of Genetics and Cytogenetics in Oncology and Haematology


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Polyclonal B Lymphocytosis with Binucleated Lymphocytes (PPBL)

Clinics and Pathology

Phenotype / cell stem origin Unknown. The polyclonal expansion of B-cells fit into the peripheral CD27+IgM+IgD+ B cell population. Cloning and sequencing of VH genes of PPBL IgVH genes showed a mutated profile suggesting like CD27 expression an expansion of memory B cells.
Etiology The etiology of polyclonal B lymphocytosis with binucleated lymphocytes (PPBL) remains unknown. An association with cigarette smoking was initially suggested. However PPBL was observed in non smokers patients. The morphology of binucleated lymphoid B-cells could suggest an association with viral infections, such as Epstein-Barr Virus. Biologic studies are not completely achieved to exclude and/or to confirm definitely the role of EBV in the pathogenesis of PPBL. The presence of characteristic binucleated lymphoid B-cells in asymptomatic family members and the description of familial PPBL cases suggest a genetic predisposition as a more likely possibility.
Epidemiology PPBL was first reported in 1982. We have no epidemiological data on the incidence of PPBL.
Clinics In a large series we reported on forty-three patients (9 males, 34 females: median age: 40 years, range 28-65), the clinical characteristics were splenomegaly in 16%, hepatomegaly in 0.5% and lymph nodes in 11.5% cases. An absolute lymphocytosis > 4 x 109/l is present in 80% of PPBL patients. A persistent, stable and polyclonal increase of IgM levels is usual and most PPBL patients express HLA-DR7. CYTOLOGY_IMAGE lymphocytosisFig1.jpg
Cytology PPBL is identified in all cases by the presence of a variable (1.5 to 9%) number of binucleated peripheral lymphoid cells (Fig 1). The majority of lymphoid cells are large with abundant faintly and basophilic cytoplasm. Characteristic nuclei with a rounded or more commonly irregular form are observed.
Immunologic markers: Both kappa and lambda light-chain are expressed, indicating a polyclonal expansion of the lymphocyte pool. The lymphocytosis is of the B-cell type: the lymphocytes react with CD19, CD20, CD22 and FMC7 antigens.
Morphologic features showing typical binucleated cells
Prognosis After a median follow-up of 5.5 years without treatment, 45 PPBL patients are alive.

Cytogenetics

Cytogenetics Morphological +i(3q) (Fig 2) is the most common abnormality and is observed in 70% cases, occurring as a single aberration in only a few patients. PCC (Fig 3) is observed in 40% cases and occur rarely as a sole abnormality. Both abnormalities associating +i(3q) and PCC are present in 37% cases.
Using alpha-satellite and telomere chromosome 3 specific probes, +i(3q) is more frequently detected by metaphase FISH studies. (Fig 2).
+i(3q) is rarely described as a recurrent cytogenetic abnormality in patients with hematologic malignancy. Trisomy 3 is reported to be associated with marginal zone B-cell lymphoma. Gain of chromosome 3 or 3q was described in patients with typical clonal b-cell chronic lymphoproliferative disorders, chronic lymphocytic leukemia, prolymphocytic leukemia or Waldenström macroglobulinemia.
A chromosomal instability is present in 67.5% patients These patients present various clonal [ del(6q), +der(8) or +8 or polyploid karyotype] and non clonal chromosomal abnormalities with structural and numerical abnormalities.
This chromosomal instability is variable over time but persist in most cases. In spite of genomic instability, a long follow-up of PPBL patients remains essential and chemotherapy unnecessary.
 
  Partial karyotype showing +i(3q) -R-banding (left); Detection of I(3q) with telomere chromosome 3q and alpha satellite specific DNA probes (right)
 
  Premature Chromosome Condensation (PCC)

Translocations implicated (Data extracted from papers in the Atlas)

Bibliography

Persistent polyclonal lymphocytosis of B lymphocytes.
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Persistent polyclonal lymphocytosis with binucleated B lymphocytes: a genetic predisposition.
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Persistent polyclonal B-cell lymphocytosis is an expansion of functional IgD(+)CD27(+) memory B cells.
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Analysis of expressed V(H) genes in persistent polyclonal B cell lymphocytosis reveals absence of selection in CD27+IgM+IgD+ memory B cells.
Loembˆ© MM, Nˆ©ron S, Delage R, Darveau A
European journal of immunology. 2002 ; 32 (12) : 3678-3688.
PMID 12516560
 
Chromosomal instability and ATR amplification gene in patients with persistent and polyclonal B-cell lymphocytosis (PPBL).
Mossafa H, Tapia S, Flandrin G, Groupe Franˆßais d'Hˆ©matologie Cellulaire (GFHC), Troussard X
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Contributor(s)

Written06-2004Xavier Troussard, Hossein Mossafa

Citation

This paper should be referenced as such :
Troussard, X ; Mossafa, H
Polyclonal B lymphocytosis with binucleated lymphocytes (PPBL)
Atlas Genet Cytogenet Oncol Haematol. 2004;8(3):248-251.
Free online version   Free pdf version   [Bibliographic record ]
URL : http://AtlasGeneticsOncology.org/Anomalies/PolyclonalLymphoID2037.html

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