Saga Tomoyuki, Ishihara Toshimichi, Ito Mariko, Kano Satoshi, Kanagawa Michiyo
Department of Hematology, Kin-ikyo Chuo Hospital.
Department of Pathology, Kin-ikyo Chuo Hospital.
Rinsho Ketsueki. 2020;61(3):251-256. doi: 10.11406/rinketsu.61.251.
Richter syndrome (RS) is the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Most cases of diffuse large B-cell lymphoma variant of RS are clonally related to the original CLL. Here, we present a case of mantle cell lymphoma (MCL) that developed sequentially during the clinical course of CLL. A 72-year-old man had been diagnosed with CLL 16 years ago and was followed-up without treatment. He developed autoimmune hemolytic anemia 2 years ago, which resolved with rituximab and prednisolone treatment. Subsequently, he presented with fever, abdominal bloating, and fatigue. Progressive lymphocytosis and splenomegaly with elevated lactic dehydrogenase levels were suggestive of RS. Bone marrow examination revealed a small- to medium-sized lymphoid infiltrate, which was positive for CD5, CD20, CCND1, and SOX-11 and negative for CD23 and LEF1 on immunostaining. Fluorescence in situ hybridization analysis was positive for IgH/CCND1, which indicated MCL. Southern blot analysis showed that both the MCL and the previous CLL expressed different IgH gene rearrangement bands. At the time of relapse or progression of CLL, sequential development of MCL should be considered.
里氏综合征（RS）是慢性淋巴细胞白血病（CLL）患者发生的侵袭性淋巴瘤。RS的弥漫性大B细胞淋巴瘤变异型的大多数病例与原始CLL存在克隆相关性。在此，我们报告一例在CLL临床过程中相继发生的套细胞淋巴瘤（MCL）病例。一名72岁男性16年前被诊断为CLL，未接受治疗并进行随访。他2年前出现自身免疫性溶血性贫血，经利妥昔单抗和泼尼松龙治疗后缓解。随后，他出现发热、腹胀和疲劳。进行性淋巴细胞增多和脾肿大伴乳酸脱氢酶水平升高提示为RS。骨髓检查发现小到中等大小的淋巴细胞浸润，免疫染色显示CD5、CD20、CCND1和SOX-11阳性，CD23和LEF1阴性。荧光原位杂交分析IgH/CCND1呈阳性，提示为MCL。Southern印迹分析显示MCL和先前的CLL均表达不同的IgH基因重排条带。在CLL复发或进展时，应考虑MCL的相继发生。
