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首页> 外文期刊>British Journal of Haematology >Hodgkin lymphoma as Richter transformation in chronic lymphocytic leukaemia: a retrospective analysis of world literature.
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Hodgkin lymphoma as Richter transformation in chronic lymphocytic leukaemia: a retrospective analysis of world literature.

机译：霍奇金淋巴瘤在慢性淋巴细胞性白血病中的Richter转化：世界文献的回顾性分析。

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摘要

Richter transformation in chronic lymphocytic leukaemia (CLL) represents an entity of considerable genetic, molecular, immunological and clinical heterogeneity. A rare occurrence, Hodgkin variant of Richter syndrome, has not been comprehensively characterized or systematized to date. We conducted a retrospective analysis of the existing cases of Hodgkin lymphoma as Richter syndrome reported in the medical literature in the previous three and a half decades. Our search identified 86 such patients; this entity affects predominantly older men and the most common histological subtype is mixed cellularity. Interval between the diagnosis of CLL and subsequent development of Hodgkin lymphoma is circa 4.3 years. The overall survival of patients was approximately 1.7 years in our analysed cohort. However, our pooled data showed that patients in whom CLL had been treated with fludarabine had a shorter survival after transformation compared to the ones not treated with this agent. The role of immunosuppression and Epstein-Barr virus infection in the aetiopathogenesis of this entity remains to be clarified.
机译：慢性淋巴细胞性白血病（CLL）的Richter转化代表了遗传，分子，免疫和临床异质性很高的一个实体。罕见的Richter综合征霍奇金病变种至今尚未得到全面表征或系统化。我们对过去三年半的医学文献中报道的霍奇金淋巴瘤如Richter综合征的现有病例进行了回顾性分析。我们的搜索确定了86位此类患者;该实体主要影响老年男性，最常见的组织学亚型是混合细胞。 CLL诊断与霍奇金淋巴瘤随后发展之间的间隔时间约为4.3年。在我们分析的队列中，患者的总生存期约为1.7年。但是，我们汇总的数据显示，与未接受该药物治疗的患者相比，接受氟达拉滨治疗的CLL患者在转化后的生存期较短。免疫抑制和爱泼斯坦-巴尔病毒感染在该个体的发病机理中的作用仍有待阐明。

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