| 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 |
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| Prognosis | After a median follow-up of 5.5 years without treatment, 45 PPBL patients are alive. |
| Persistent polyclonal lymphocytosis of B lymphocytes. |
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