摘要目的 探讨原发心脏CD5阳性且为bcl-2、C-MYC双表达弥漫性大B细胞淋巴瘤的临床病理特征及治疗和预后情况.方法 收集广东省人民医院诊治的心脏CD5阳性且为bcl-2、C-MYC双表达弥漫性大B细胞淋巴瘤患者2例的临床信息,分析其组织学形态、免疫表型、荧光原位杂交及治疗和预后情况.结果 临床特征:例1男性患者,年龄55岁;例2女性患者,年龄61岁.手术所见:右心房或心室巨大肿物并侵犯邻近组织.组织学形态:形态较单一的大淋巴样细胞弥漫分布,核分裂象及凋亡易见.免疫组织化学结果:肿瘤细胞弥漫表达CD20、CD5、bcl-6、MUM1、C-MYC及bcl-2,Ki-67阳性指数≥90%.C-MYC、bcl-2及bcl-6荧光原位杂交:例1患者发生bcl-6易位,无其余基因异常;例2无基因异常.治疗及预后:例1患者经手术切除肿物确诊后放弃化疗,1个月后死亡;例2患者经4疗程的R-CHOP方案(利妥昔单抗+环磷酰胺+多柔比星+长春新碱+泼尼松龙)治疗后病情好转,改用阿帕替尼及依鲁替尼联合口服治疗,随访18个月病情稳定.结论 原发心脏CD5阳性且bcl-2、C-MYC双表达弥漫性大B细胞淋巴瘤常表现为右心房或心室浸润性生长的大肿块、非生发中心B细胞免疫表型亚群并具有很高的增殖指数,可能提示了一部分原发心脏侵袭性淋巴瘤的病理特征.

abstractsObjectives To investigate the clinicopathological features, therapy and prognosis of primary cardiac CD5-positive diffuse large B-cell lymphoma with C-MYC and bcl-2 double expression. Methods Two cases diagnosed at Guangdong Provincial People′s Hospital were included, the clinical data were collected; the tumor morphology, immunophenotypic profiles, therapy and prognosis were analyzed. Results Case 1 was a 55-year-old man and case 2 was a 61-year-old women. Intraoperatively, both cases showed large masses in the right atrium or ventricle, involving adjacent tissue. Pathologically, the tumors were composed of diffusely infiltrating large lymphoid cells with high mitotic activity and apoptosis. The tumor cells were positive for CD20, CD5, bcl-6, MUM1, C-MYC and bcl-2, and the Ki-67 index was equal or greater than 90%. Case 1 had bcl-6, but not bcl-2 or MYC gene rearrangements. No MYC, bcl-2 or bcl-6 gene rearrangements were detected in case 2. Case 1 defaulted chemotherapy after operation and died 1 month after diagnosis. Case 2 was treated with 4 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) therapy after surgery and attained partial remission, and was then treated with apatinib and ibrutinib, and remained stable 18 months after initial diagnosis. Conclusion Primary cardiac CD5-positive diffuse large B-cell lymphoma with C-MYC and bcl-2 double expression usually shows large infiltrative mass in the right atrium or ventricle, non-germinal center like immunophenotype and high proliferation index, and this may contribute to the aggressiveness of primary cardiac lymphoma.