Hodgkin lymphoma as Richter transformation in chronic lymphocytic leukaemia: a retrospective analysis of world literature
Hodgkin lymphoma as Richter transformation in chronic lymphocytic leukaemia: a retrospective analysis of world literature
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DOI：
10.1111/j.1365-2141.2011.08907.x
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发表时间：
2012-01-01
影响因子：
6.5
通讯作者：
Dasanu, Constantin A.
中科院分区：
文献类型：
--
作者：
Bockorny, Bruno;Codreanu, Ion;Dasanu, Constantin A.
慢性淋巴细胞白血病（CLL）的 Richter转化在遗传、分子、免疫和临床方面具有相当大的异质性。Richter综合征的霍奇金变异型较为罕见，迄今为止尚未得到全面的特征描述或系统化。我们对过去35年医学文献中报道的Richter综合征中的霍奇金淋巴瘤现有病例进行了回顾性分析。我们的检索确定了86例此类患者；这种情况主要影响老年男性，最常见的组织学亚型是混合细胞型。慢性淋巴细胞白血病诊断与随后霍奇金淋巴瘤发生之间的间隔约为4.3年。在我们分析的队列中，患者的总体生存期约为1.7年。然而，我们汇总的数据显示，与未使用氟达拉滨治疗的患者相比，使用氟达拉滨治疗慢性淋巴细胞白血病的患者在转化后的生存期更短。免疫抑制和爱泼斯坦 - 巴尔病毒感染在这种疾病的发病机制中的作用仍有待阐明。
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 EpsteinBarr virus infection in the aetiopathogenesis of this entity remains to be clarified.