霍奇金变异型里氏综合征 (HvRS) 是一种罕见的并发症，发生于 1% 的淋巴细胞淋巴瘤/慢性淋巴细胞白血病患者。我们报告一例在撒哈拉以南非洲地区诊断出的 HvRS 病例。一名 63 岁患者因腹部肿块的检查而接受会诊，该肿块在入院前已经发展了 5 年。他的病史显示盗汗、3 个月内体重减轻 13% 以及持续性瘙痒。检查发现双侧颈腋窝和腹股沟大腺病、无痛性腹胀、瘙痒性病变和 WHO 2 PS。血细胞计数显示贫血为 9.5 g/dL。组织学显示弥漫性结构的淋巴瘤增殖，局部呈结节状，霍奇金细胞和斯滕伯格细胞与小淋巴细胞、组织细胞和嗜酸性多形性相关。免疫组化显示CD20、PAX5、BCL2、CD5、CD23和MYC阳性； Ki67 为 10%，细胞周期蛋白 D1、BCL6 和 CD10 阴性；霍奇金和斯滕伯格细胞 CD30 呈阳性，但 CD20 呈阴性；解读 CD15 存在困难； EBV 阳性 (EBER)；反应性 T 细胞上 CD3 和 CD5 呈阳性。 CD138 以及 kappa 和 lambda 轻链是非贡献性的。扩展检查将患者分类为安娜堡 III B 期，Hasenclever 评分为 3/7。这个案例说明了我国血液病理学家数量不足、技术设施有限，诊断HvRS的困难。

The Hodgkin variant Richter syndrome (HvRS) is an infrequent complication occurring in 1% of lymphocytic lymphoma/chronic lymphocytic leukemia patients. We report a case of HvRS diagnosed in Sub-Saharan Africa. A 63-year-old patient was consulted for the investigation of an abdominal mass that had been evolving for 5 years prior to admission. His history revealed night sweats, 13% weight loss in 3 months and persistent pruritis. Examination revealed bilateral cervical axillary and inguinal macroadenopathies, painless abdominal distension, pruritic lesions and WHO 2 PS. The blood count showed anemia at 9.5 g/dL. Histology revealed a lymphomatous proliferation of diffuse architecture, nodular in places, with Hodgkin and Sternberg cells associated with small lymphocytes, histiocytes and eosinophilic polymorphs. Immunohistochemistry showed CD20, PAX5, BCL2, CD5, CD23 and MYC positivity; Ki67 at 10% and cyclin D1, BCL6 and CD10 negativity; CD30 positivity on Hodgkin and Sternberg cells that remained CD20 negative; difficulty interpreting CD15; EBV positivity (EBERs); and CD3 and CD5 positivity on reactive T cells. CD138 and kappa and lambda light chains were non-contributory. The extension work-up classified the patient as Ann Arbor stage III B with a Hasenclever score of 3/7. This case illustrates the difficulties in diagnosing HvRS in our countries, where the number of haematopathologists is insufficient and the technical facilities are limited.