Touré Sokhna Aïssatou, Niang Dibor, Gueye Serigne Mourtalla, Keita Mohamed, Diallo Alioune Badara, Bousso Elimane Seydi, Dieng Fatma, Faye Blaise Felix, Seck Moussa, Diop Saliou
Hematology Department, Cheikh Anta Diop University, Dakar 10700, Senegal.
Pathology Department, Gaston Berger University, Saint-Louis 234, Senegal.
Hematol Rep. 2024 Aug 5;16(3):523-528. doi: 10.3390/hematolrep16030050.
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.
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中。我们报告一例在撒哈拉以南非洲诊断出的HvRS病例。一名63岁患者因腹部肿块就诊,该肿块在入院前已发展5年。他的病史显示有盗汗、3个月内体重减轻13%以及持续性瘙痒。检查发现双侧颈部、腋窝和腹股沟淋巴结肿大、无痛性腹胀、瘙痒性皮损以及世界卫生组织2级体能状态。血常规显示贫血,血红蛋白为9.5g/dL。组织学检查显示为弥漫性结构的淋巴瘤样增生,部分呈结节状,伴有霍奇金细胞和施特恩贝格细胞,以及小淋巴细胞、组织细胞和嗜酸性多形核细胞。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;霍奇金细胞和施特恩贝格细胞CD30阳性,但仍CD20阴性;CD15难以判读;EBV阳性(EBERs);反应性T细胞CD3和CD5阳性。CD138以及κ和λ轻链无诊断意义。分期检查将该患者归类为Ann Arbor分期III B期,Hasenclever评分为(3/7)。该病例说明了在我们国家诊断HvRS的困难,这里血液病理学家数量不足且技术设施有限。