Hematology Department and HCT Unit, G. Papanicolaou Hospital, Thessaloniki, Greece.
Mediterr J Hematol Infect Dis. 2012;4(1):e2012052. doi: 10.4084/MJHID.2012.052. Epub 2012 Aug 9.
Immunogenetic analysis of the B cell receptors (BCRs) has been a richly rewarding field for unraveling the pathogenesis of human lymphomas, including CLL. A biased immunoglobulin gene repertoire is seen as evidence for selection of CLL progenitor cells by antigen. Additional corroborative evidence is provided by the differential prognosis of cases with distinct mutational status of the clonotypic BCRs. However, perhaps the strongest immunogenetic evidence for the importance of interactions with microenvironment in driving CLL development and evolution is the existence of subsets of patients with quasi-identical, stereotyped BCRs, collectively accounting for a remarkable one-third of the entire cohort. These observations have been instrumental in shaping the notion that CLL ontogeny is functionally driven and dynamic, rather than a simple stochastic process. From a clinical perspective, ample evidence indicates that immunogenetic information can be used for the biologically and clinically rational categorization of CLL, with important potential implications for basic, translational and clinical research.
免疫遗传学分析 B 细胞受体（BCRs）一直是揭示人类淋巴瘤（包括 CLL）发病机制的一个非常有价值的领域。BCR 免疫球蛋白基因库的偏倚被认为是 CLL 祖细胞受抗原选择的证据。另外，不同突变状态的克隆型 BCR 病例具有不同的预后，这为其提供了额外的佐证。然而，也许与微环境相互作用在推动 CLL 发展和演变方面最重要的免疫遗传学证据是存在具有准相同、刻板 BCR 的亚组患者，这些患者占整个队列的惊人的三分之一。这些观察结果对于塑造 CLL 发生是功能驱动和动态的，而不是简单的随机过程的概念起到了重要作用。从临床角度来看，大量证据表明免疫遗传学信息可用于对 CLL 进行生物学和临床合理分类，这对基础、转化和临床研究具有重要的潜在意义。
