Callet-Bauchu E, Renard N, Gazzo S, Poncet C, Morel D, Pagès J, Salles G, Coeur P, Felman P
Laboratoire Central d'Hématologie, Centre Hospitalier Lyon Sud, Pierre-Bénite, France.
Br J Haematol. 1997 Dec;99(3):531-6. doi: 10.1046/j.1365-2141.1997.4233234.x.
Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare entity characterized by a moderate but sustained lymphocytosis where some binucleated or bilobulated circulating forms constitute, even if they are not entirely specific, the cytological hallmark of the disease. An additional chromosome long arm i(3)(q10) has recently been reported as a recurrent cytogenetic aberration, contrasting with a usual polyclonal immunoglobulin expression. To determine more precisely the distribution of the chromosomal abnormality within the peripheral lymphocyte population and study the relationship between the +i(3)(q10) and the bilobulated character, we investigated three new cases of PPBL displaying the cytogenetic abnormality on the karyotype, using a technique of simultaneous fluorescence immunophenotyping and interphase cytogenetics (FICTION). We demonstrated that the +i(3)(q10) was restricted to the B lymphocytes, independently of the kappa or lambda light chain isotype and was present in both bilobulated and non-bilobulated cells. Therefore it is likely that the cytogenetic abnormality occurs at an early stage of lymphocyte differentiation in a precursor cell already committed to the B-cell lineage, before any rearrangement of immunoglobulin genes has taken place.
持续性多克隆B细胞淋巴细胞增多症(PPBL)是一种罕见的病症,其特征为淋巴细胞中度但持续增多,其中一些双核或双叶循环形式构成了该病的细胞学特征,即便它们并非完全特异。最近有报道称额外的3号染色体长臂i(3)(q10)是一种反复出现的细胞遗传学异常,这与通常的多克隆免疫球蛋白表达情况形成对比。为了更精确地确定外周淋巴细胞群体中染色体异常的分布,并研究+i(3)(q10)与双叶特征之间的关系,我们使用同步荧光免疫表型分析和间期细胞遗传学技术(FICTION),对3例核型显示细胞遗传学异常的PPBL新病例进行了研究。我们证明+i(3)(q10)仅限于B淋巴细胞,与κ或λ轻链同种型无关,且存在于双叶和非双叶细胞中。因此,细胞遗传学异常很可能发生在已定向于B细胞系的前体细胞的淋巴细胞分化早期阶段,此时免疫球蛋白基因尚未发生任何重排。