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Detection of monoclonal plasma cells in bone marrow and spleen of primary amyloidosis

机译:原发性淀粉样变性的骨髓和脾脏中单克隆浆细胞的检测

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A 53-year-old man was diagnosed as primary amyloidosis by biopsy specimens of the mucosa in rectum, spleen and bone marrow (BM). BM examination showed 5.5% of plasma cells with some dysplasia. Immunoglobulin heavy chain gene rearrangement was detected by polymerase chain reaction using third-complementary-determining region (CDR 3) specific primers in BM mononuclear cells and spleen cells embedded in paraffin. The sequence analysis revealed that monoclonal B cells existed in the both, BM and spleen. Flowcytometric analysis using two-color staining showed the phenotype of plasma cells with the expression of CD19+/-, CD27+/-, CD56+/- and CD138+ and CD38++. This phenotype is similar to those of monoclonal gammopathy of undetermined significance (MGUS). Therefore, primary amyloidosis is considered to have two plasma cell populations including normal plasma cells and monoclonal plasma cells in BM. Given that there are two types of plasma cells in patients with primary amyloidosis, amyloid protein is expected to originate from immunoglobulin light chain produced by monoclonal plasma cells.
机译:通过活组织检查直肠,脾脏和骨髓(BM)的粘膜标本,将一名53岁的男性诊断为原发性淀粉样变性。 BM检查显示5.5%的浆细胞有不典型增生。免疫球蛋白重链基因重排通过聚合酶链反应使用BM单核细胞和石蜡包埋的脾细胞中的第三互补决定区(CDR 3)特异性引物进行检测。序列分析表明,BM和脾脏中均存在单克隆B细胞。使用双色染色的流式细胞术分析显示浆细胞的表型,其表达为CD19 +/-,CD27 +/-,CD56 +/-,CD138 +和CD38 ++。该表型与意义不明的单克隆丙种球蛋白病(MGUS)相似。因此,原发性淀粉样变性被认为具有两个浆细胞群,包括正常浆细胞和BM中的单克隆浆细胞。考虑到原发性淀粉样变性患者中浆细胞有两种类型,因此淀粉样蛋白有望源自单克隆浆细胞产生的免疫球蛋白轻链。

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