Persistent polyclonal B-cell lymphocytosis: study of 35 cases

• Published in: Medicina clinica Vol. 136; no. 13; p. 565
• Main Authors: Florensa, Lourdes, Navarro, José Tomás, Pérez Vila, María Encarnación, Domingo, Alicia, De la Banda, Esmeralda, Rozman, Maria, Camós, Mireia, Millá, Fuensanta, Perea, Granada, Alonso, Esther, Ayats, Ramon, Aventín, Ana, Cabezudo, Elena, Espinet, Blanca, Merino, Ana, Romero, Pilar, Sánchez, Carmen, Tuset, Esperanza, Solé, Francesc, Feliu, Evarist, Fernández, Cristalina, Gallart, Miquel, Vallespí, Teresa, Woessner, Soledad
• Format: Journal Article
• Language: Spanish
• Published: Spain 14.05.2011
• Subjects: Adult; B-Lymphocytes - metabolism; B-Lymphocytes - pathology; Disease Progression; Female; Follow-Up Studies; Humans; Kaplan-Meier Estimate; Lymphocyte Activation; Lymphocytosis - genetics; Lymphocytosis - immunology; Lymphocytosis - mortality; Male; Middle Aged; Precancerous Conditions - immunology; Retrospective Studies; Sex Distribution; Smoking
• ISSN: 1578-8989
• DOI: 10.1016/j.medcli.2010.09.048

Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare entity, presenting especially in adult smoker women. It is characterized by an increase of serum IgM, DR7-HLA haplotype, cytogenetic abnormalities and multiple IgH/BCL-2 rearrangements. To date, it has not been elucidated whether this is a benign or premalignant disorder. We analyzed the PPBL characteristics with especial attention to its evolution. Thirty-five PPBL patients from 5 hospitals in Catalonia were retrospectively analyzed. A simultaneous morphologic review of the blood smears was performed by members of the GCCH in a 16 multiple-observer optic microscope. Clinical and biological data were also analyzed. PPBL presents in the majority of cases with persistent polyclonal B-cell lymphocytosis and affects primarily smoker women. The morphologic hallmark, in absence of viral infections, is the presence of activated lymphocytes with bilobulated and/or cleaved nuclei, and nuclear pockets in the ultrastructural study. Increased serum IgM, HLA-DR7 haplotype, chromosomal abnormalities such as i(3)(q10) and multiple IgH/BCL-2 rearrangements were detected. Thirty-four out of 35 patients are alive after a median follow up of 70.7 months. One patient died because of lung adenocarcinoma and another developed a follicular lymphoma without relation to PPBL. PPBL has an asymptomatic and stable evolution, although it frequently presents genetic abnormalities. It remains unknown whether it is a premalignant entity, similar to monoclonal gammopathies of unknown significance. Hence, accurate cytologic diagnosis and follow-up are essential.