10例B细胞淋巴瘤相关噬血细胞综合征的临床分析

发布时间：2018-05-24 21:46

本文选题：淋巴瘤 + B细胞淋巴瘤　；参考：《中国实验血液学杂志》2017年04期

【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。
[Abstract]:Objective: To investigate the clinical and laboratory characteristics of B cell lymphoma related hemophagocytic syndrome (B-LAHS). Methods: retrospective analysis of the clinical data of 10 patients with B-LAHS and review the relevant literature. Results: the median age of 10 patients was 55.5 (31-88) years, from the onset to the median time of February (2 weeks -4 months). 7 cases of large B cell lymphoma, 2 cases of NHL and 1 cases of small B cell lymphoma that could not be classified, were diagnosed and immunized by immunohistochemistry. The clinical manifestations were persistent fever (100%) and splenomegaly (90%), while respiratory and digestive system involvement was common, and 1 cases were first presented with systemic myalgia and lactic acidosis. The room examination showed that there were different degrees of liver function damage, significant ferritin and lactate dehydrogenase increased, peripheral blood smears found abnormal lymphocytes, bone marrow smears were easy to see hemophagocytic phenomenon. Flow cytometry detected the abnormal forward scattering / laterally scattered light (FSC/SSC) of light chain restrictive B lymphoma cells in.4 cases received ritubitus The patients with celecoxib based immuno chemotherapy maintained complete remission (CR) at the date of follow-up. Conclusion: the clinical difference of B-LAHS is great, the rapid progress of the disease, the histopathology of bone marrow biopsy and immunohistochemical examination can be clearly diagnosed. The immunophenotype analysis of flow cytometry can improve the early diagnosis of B-LAHS.
【作者单位】：北京大学深圳医院血液内科;
【基金】：2012年深圳市科技计划项目(201203030)
【分类号】：R55;R733.1

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