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Long-term follow-up of 111 patients with persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes

Leukemia volume 23, pages 419–422 (2009)Cite this article

Initially described in 1982, the persistent polyclonal B-cell lymphocytosis (PPBL) is characterized by a chronic, stable, persistent and polyclonal lymphocytosis, the presence of binucleated lymphocytes in the peripheral blood and a polyclonal increase in serum immunoglobulin-M (IgM).1 In this apparently benign entity, we showed that PPBL was associated with recurrent chromosomal abnormalities and a typical cytogenetic profile including isochromosome 3q, +i(3q), premature chromosome condensation (PCC), both abnormalities in the same patient or chromosomal instability.2 Despite clinical and polyclonal lymphocytosis stability, the long-term follow-up is not yet well established.

We analyze and report here the long-term follow-up of 111 patients with typical PPBL. PPBL was diagnosed in 91 women (82%) (median age 40 years, range 19–66 years) and 20 men (median age 40.9 years, range 28–57 years). The patients, smokers in 98% of cases, were either asymptomatic or had minor and nonspecific complaints, such as fatigue. Physical examination was usually within normal limits, except for a mild splenomegaly present in 10% of cases. PPBL was diagnosed when blood smear analysis revealed the presence of a variable number of binucleated lymphocytes, whatever the lymphocyte count. At the time of diagnosis, 83% of patients presented with mild lymphocytosis and 17% with a lymphocyte count below 4 × 109/l. The median lymphocyte count was 5.45 × 109/l, with a range of 2.45–20.52 × 109/l. In all cases, peripheral blood examination showed typical binucleated lymphocytes, which were present with a variable percentage (median 4%, range 1–40%). Peripheral blood flow cytometry showed a polyclonal B-cell lymphocytosis in all cases, with an expansion of the B CD19-positive and CD5-negative lymphocytes pool. Both kappa and lambda light chain were expressed in all cases, confirming the B-cell polyclonal expansion. The median of polyclonal serum IgM level was 6.0 g/l (normal: 0.5–2.7 g/l), with a range of 1.2–17.3 g/l. Ninety-eight patients underwent conventional cytogenetic analysis (CCA). Isochromosome +i(3q) was present in 34% (33 of 98 patients), PCC in 8% (8 of 98 patients) and both abnormalities in 31% (30 of 98 patients). CCA showed neither +i(3q) nor PCC in 28% (27 of 98 patients). Fluorescence in situ hybridization (FISH) was performed in 84 cases. Isochromosome +i(3q) was studied in interphase with alpha-satellite chromosome 3-specific (SO CEP3) and LSI 3′BCL6 (3q27) break-apart probes obtained from Abott Molecular (Rungis, France). Isochromosome +i(3q) was detected in 71% (60 of 84 patients). We also combined CCA and FISH in 84 patients: after FISH, +i(3q) was detected in 17 patients with negative CCA and was confirmed in 43 patients with positive CCA. CCA and FISH were both negative in 24 cases.

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Authors and Affiliations

  1. Laboratoire d’Hématologie, CHU de Caen, Registre Régional des Hémopathies Malignes de Basse-Normandie and UPRES EA 3919, Caen, France

    E Cornet & X Troussard

  2. Laboratoire d’Hématologie Biologique, CHU de Nancy, Vandoeuvre, France

    J F Lesesve

  3. Laboratoire Pasteur-Cerba, Laboratoire de Cytogénétique, Cergy-Pontoise, France

    H Mossafa

  4. Laboratoire d’Hématologie, Hôpital Nord, Marseille, France

    G Sébahoun

  5. CIC 9504, Hôpital Saint Louis, APHP, Université Paris 7 and INSERM U717, Paris, France

    V Levy

  6. Laboratoire d’Hématologie, Hôpital Pitié-Salpêtrière et Université Pierre et Marie Curie, Paris, France

    F Davi

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Cornet, E., Lesesve, J., Mossafa, H. et al. Long-term follow-up of 111 patients with persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes. Leukemia 23, 419–422 (2009). https://doi.org/10.1038/leu.2008.208

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