• 作者：Lourdes Florensa^a ; ^b ; ^o ; ^{lflorensa@imas.imim.es} ; José ; Tom&#225 ; s Navarro^c ; ^o ; Marí ; a Encarnació ; n Pé ; rez Vila^a ; ^b ; ^o ; Alicia Domingo^d ; ^o ; Esmeralda De la Banda^d ; ^o ; Maria Rozman^e ; ^o ; Mireia Camó ; s^e ; ^o ; Fuensanta Mill&#225 ; ^c ; ^o ; Granada Perea^f ; ^o ; Esther Alonso^d ; ^o ; Ramon Ayats^g ; ^o ; Ana Aventí ; n^g ; ^o ; Elena Cabezudo^h ; ^o ; Blanca Espinet^b ; ⁱ ; Ana Merino^j ; ^o ; Pilar Romero^k ; ^o ; Carmen S&#225 ; nchez^l ; ^o ; Esperanza Tuset^m ; ^o ; Francesc Solé ; ^b ; ⁱ ; Evarist Feliu^c ; ^o ; Cristalina Fern&#225 ; ndezⁿ ; ^o ; Miquel Gallart^ñ ; ; ^o ; Teresa Vallespí ; ^l ; ^o ; Soledad Woessner^b ; ^o
• 关键词：Linfocitosis B policlonal ; Fumador ; Linfocito bilobulado ; HLA-DR7 ; i(3)(q10)
• 刊名：Medicina Cl铆nica
• 出版年：2011
• 期刊代码：pca254_00257753
• 类别：med
• 出版时间：14 May 2011
• 卷：136
• 期：13
• 页码：565-573
• 文件大小：645 K

Background and objectives

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.

Patients and methods

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.

Results

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.

Conclusions

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.