10例B细胞淋巴瘤相关噬血细胞综合征的临床分析  

Clinical Analysis of 10 Cases of B Cell Lymphoma-Associated Hemophagocytic Syndrome

作　　者：钟凤鸾[1] 张红宇[1] 张倩[1] 张文丽[1] 冯佳[1] 孟庆祥[1] 

机构地区：[1]北京大学深圳医院血液内科,广东深圳518036

出　　处：《中国实验血液学杂志》2017年第4期1022-1029,共8页Journal of Experimental Hematology

基　　金：2012年深圳市科技计划项目(201203030)

摘　　要：目的:探讨B细胞淋巴瘤相关噬血细胞综合征(B-LAHS)的临床和实验室特征。方法:回顾性分析10例B-LAHS患者的临床资料,并复习相关文献。结果:10例患者确诊时的中位年龄55.5(31-88)岁,自起病至确诊的中位时间2月(2周-4月)。经骨髓活检组织病理及免疫组化确诊大B细胞淋巴瘤7例,套细胞淋巴瘤2例,不能分类的小B细胞淋巴瘤1例。临床均以持续性发热(100%)和脾肿大(90%)为突出表现,而呼吸系统受累和消化系统受累表现为常见,以全身的肌痛和乳酸性酸中毒为首发表现1例;实验室检查显示有不同程度的肝功能损害、显著的铁蛋白和乳酸脱氢酶升高,外周血涂片发现异常的淋巴细胞,骨髓涂片易见噬血细胞现象,流式细胞仪均检测到异常前向散射/侧向散射光(FSC/SSC)的呈轻链限制性B淋巴瘤细胞。4例接受以利妥昔单抗为基础的免疫化疗患者截至随访日期维持完全缓解(CR)。结论:B-LAHS临床差异极大,疾病快速进展,骨髓活检组织病理及免疫组织化学检查可明确诊断,流式细胞仪的免疫表型分析可改善B-LAHS的早期诊断。Objective： To investigate the clinical and laboratory features of patient with B cell lymphoma associated hemophagocytic syndrome（ B-LAHS）. Methods： The clinical data of 10 cases of B-LAHS were retrospectively analysed and the relevant literatures were reviewed. Results： The median age of 10 cases diagnosed as B-LAHS was 55. 5（ 31-88） years old,and median time from attack to diagnosis was 2 months（ 2 weeks-4 months）. The diagnosis can be made histopathologically and immunohistochemically by bone marrow biopsy. Among them 7 cases were diagnosed as large B cell lymphoma,2 cases as mantle cell lymphoma and 1 case as small B cell lymphoma. The prominent clinical symptoms and signs were persistent fever（ 100%） and splenomegaly（ 90%）,and the involvements with respiratory and digestive system were common. Another 1 case had systemic muscle pain and lactic acidosis as the first onset.Laboratory studies showed hepatic dysfunction,significantly elevated ferritin and lactate dehydrogenase,abnormal lymphocytes in peripheral blood smear,and hemophagocytosis in bone marrow smear. The FSC/SSC abnormalities of cloned B lymphoma cells were detected through flow cytometry（ FCM）. The complete remission（ CR） was maintained in 4 cases receiving immunochemotherapy based on rituximab. Conclusion： B-LAHS possesses heterogeneous clinical manifestations and rapid deterioration. Bone marrow biopsy and immunohistochemical examination can confirm the diagnosis. FCM may improve the early diagnosis of B-LAHS.

关 键 词：淋巴瘤 B细胞淋巴瘤 噬血细胞综合征 B细胞淋巴瘤相关噬血细胞综合征 

分 类 号：R733.1[医药卫生—肿瘤]