Dujardin Fanny, Lefrancq Thierry, Bléchet Claire, Boni-Boka Michel, Sénecal Delphine, Desmoulins Isabelle, Guyétant Serge
Service d'anatomie et de cytologie pathologiques, hôpital Trousseau, CHRU de Tours, 37044 Tours cedex 09, France.
Ann Pathol. 2008 Sep;28(4):311-6. doi: 10.1016/j.annpat.2007.11.009. Epub 2008 Aug 26.
We report the clinical and immunohistological features of two cases of chronic lymphocytic leukaemia (CLL) with Hodgkin's transformation. These cases occurred in a 70-year-old man with a three-year history of CLL and in a 76-year-old man with a few months history of CLL. Microscopic examination showed the presence of large tumor cells with the morphological and immunophenotypic features of classical Hodgkin and Reed-Sternberg (R-S) cells, in a background of otherwise typical B-CLL. The transformation of CLL into large B cell lymphoma (Richter's syndrome) is a well-documented phenomenon. Only rarely does CLL transform into Hodgkin's lymphoma, but this diagnosis is often easy and offers few differential diagnoses. The major points of interest lie in the pathogenetic relationship between CLL and Hodgkin's disease, and in the potential clinical implications of this peculiar condition. Literature on the subject indicates that identical IgH gene rearrangements in micromanipulated R-S and CLL cells have been identified in 7/12 cases. In these patients, the R-S and CLL cells belong to the same clonal population, suggesting a progression from the underlying CLL cells. This group appears to have a poor prognosis, identical to classical Richter's syndrome. In other cases, the R-S cells were often Epstein-Barr virus (EBV) positive and did not share the clonal rearrangements identified in CLL cells, suggesting that Hodgkin's disease in these patients could represent a second malignancy, EBV-related and favored by immunosuppression, associated with a better prognosis.
我们报告了两例慢性淋巴细胞白血病（CLL）发生霍奇金转化的临床和免疫组织学特征。这两例分别发生在一名有3年CLL病史的70岁男性和一名有几个月CLL病史的76岁男性身上。显微镜检查显示，在典型B-CLL背景中存在具有经典霍奇金和里德-斯腾伯格（R-S）细胞形态及免疫表型特征的大肿瘤细胞。CLL转化为大B细胞淋巴瘤（ Richter综合征）是一种有充分文献记载的现象。CLL很少转化为霍奇金淋巴瘤，但这种诊断通常容易，且鉴别诊断较少。主要关注点在于CLL与霍奇金病之间的发病机制关系，以及这种特殊情况的潜在临床意义。关于该主题的文献表明，在12例中的7例中，在显微操作的R-S细胞和CLL细胞中鉴定出相同的IgH基因重排。在这些患者中，R-S细胞和CLL细胞属于同一克隆群体，提示由潜在的CLL细胞进展而来。该组患者预后似乎较差，与经典的Richter综合征相同。在其他病例中，R-S细胞常为EB病毒（EBV）阳性，且不具有在CLL细胞中鉴定出的克隆重排，提示这些患者的霍奇金病可能代表第二种恶性肿瘤，与EBV相关且受免疫抑制影响，预后较好。
