检测骨髓异常浆细胞有助于诊断原发性系统性轻链淀粉样变性  

Detection of Abnormal Plasma Cells in Bone Marrow Contributes to the Diagnosis of Primary Systemic Light Chain Amyloidosis——Review

作　　者：胡旸[1] 朱平[1] 

机构地区：[1]北京大学第一医院血液研究室,北京100034

出　　处：《中国实验血液学杂志》2012年第5期1251-1255,共5页Journal of Experimental Hematology

摘　　要：原发性系统性轻链淀粉样变性(AL)是系统性淀粉样变性疾病中最常见的类型,最近已明确AL是异常浆细胞导致的恶性肿瘤。这些浆细胞产生过量的免疫球蛋白轻链,形成具有蛋白质毒性的纤维状淀粉样物,通过异常折叠和沉积于多种组织器官,引起多脏器损害。AL诊断较困难,以往主要通过活检组织的刚果红染色。现在使用流式细胞术可以通过患者骨髓浆细胞的免疫球蛋白轻链限制性及异常表型特征检测异常浆细胞克隆,有助于早期确诊AL,并作为诊断AL后临床疗效监测的重要指标。本文就AL的诊断和分型、临床特点、骨髓浆细胞免疫表型的流式细胞术检测,异常浆细胞免疫球蛋白轻链限制性和表型特点等作一简要的综述。Primary systemic light chain amyloidosis or immunoglobulin light-chain amyloidosis（AL） is the most common type of systemic amyloidosis.AL is a proteotoxic clonal plasma cell disease,a hematological malignancy,characterised by overproduction of immunoglobulin light chains that form characteristic abnormally folded and aggregated,insoluble fibrillar deposits in various organs,including kidneys,heart,liver,and autonomic and peripheral nerves,etc,these processes lead to organ dysfunction and death.Systemic amyloidosis have various types with different causes,thereby its clinical diagnosis and treatment are more difficult.Recent developments on studies that have significantly aided the management of patients with AL include diagnostic techniques for definitive typing of amyloid deposits by using flow cytometry and immunophenotype analysis.These methods can detect abnormalities of bone marrow plasma cell clones,such as CD38＋,CD138＋,CD56＋,CD19-in AL patients.The monitoring abnormal plasma cells with immunoglobulin light chain restriction and abnormal plasma cell phenotypic characteristics contributes to the early diagnosis of AL and detection of minimal residual disease after treatment,which greatly improved AL treatment and prognosis.In this review the diagnosis and typing,clinical characteristics,flow cytometry,immunophemtyping of bone marrow cells,immunoplobulin light chain restriction and phenotypic characteristics of abnormal plasma cells of AL are briefly summaried.

关 键 词：原发性系统性轻链淀粉样变性 系统性淀粉样变性 流式细胞术 免疫表型 轻链限制性 

分 类 号：R551[医药卫生—血液循环系统疾病]