Mossafa H, Malaure H, Maynadie M, Valensi F, Schillinger F, Garand R, Jung G, Flandrin G, Troussard X
Laboratoire Cerba, Cergy-Pontoise, France.
Br J Haematol. 1999 Mar;104(3):486-93. doi: 10.1046/j.1365-2141.1999.01200.x.
Persistent and polyclonal lymphocytosis of B lymphocytes (PPBL) with binucleated lymphocytes is an entity characterized by a polyclonal lymphocytosis. The lymphocytosis is stable for years and binucleated lymphocytes are detected on peripheral blood smears. We previously described +i(3q) as a recurrent chromosomal abnormality in seven PPBL patients. In this study we report a large series of 25 PPBL patients and demonstrated that PPBL was associated with +i(3q) in 77% of cases, premature chromosome condensation (PCC) in 50% and both abnormalities in 41% of cases. Furthermore, we demonstrated that i(3q) was present in a minority of B cells, restricted to B lymphocytes independently of the kappa or lambda light Ig chain expression, and exclusively observed in non-binucleated cells. The benign clinical course of PPBL and the lack of biological evolution in the majority of cases suggest that recognition of these disorders is so important that aggressive therapy in PPBL has to be avoided. Whether this syndrome represents a premalignant or benign disease remains unclear. The persistence of cytogenetic abnormalities after stopping tobacco use suggests no association with cigarette smoking.
伴有双核淋巴细胞的B淋巴细胞持续性多克隆淋巴细胞增多症(PPBL)是一种以多克隆淋巴细胞增多为特征的疾病。淋巴细胞增多可持续数年,在外周血涂片上可检测到双核淋巴细胞。我们之前报道了+i(3q)是7例PPBL患者中反复出现的染色体异常。在本研究中,我们报告了一大组25例PPBL患者,并证明77%的病例中PPBL与+i(3q)相关,50%的病例与染色体早熟凝集(PCC)相关,41%的病例同时存在这两种异常。此外,我们证明i(3q)存在于少数B细胞中,独立于κ或λ轻链免疫球蛋白表达,仅限于B淋巴细胞,且仅在非双核细胞中观察到。PPBL的良性临床病程以及大多数病例缺乏生物学演变表明,认识这些疾病非常重要,必须避免对PPBL进行积极治疗。该综合征是代表癌前病变还是良性疾病仍不清楚。戒烟后细胞遗传学异常持续存在,提示与吸烟无关。