Katayama Y, Sakai A, Nobuyoshi M, Shimomura T, Kimura A
Department of Hematology and Oncology, Division of Clinical Research, Research Institute for Radiation Biology and Medicine, Hiroshima University.
Rinsho Ketsueki. 2001 Sep;42(9):705-9.
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.
一名53岁男性经直肠、脾脏和骨髓（BM）黏膜活检标本诊断为原发性淀粉样变性。骨髓检查显示5.5%的浆细胞有一定发育异常。使用针对第三互补决定区（CDR 3）的特异性引物，通过聚合酶链反应在石蜡包埋的骨髓单个核细胞和脾脏细胞中检测到免疫球蛋白重链基因重排。序列分析显示骨髓和脾脏中均存在单克隆B细胞。双色染色的流式细胞术分析显示浆细胞表型为CD19+/-、CD27+/-、CD56+/-、CD138+以及CD38++。该表型与意义未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在骨髓中有包括正常浆细胞和单克隆浆细胞在内的两种浆细胞群。鉴于原发性淀粉样变性患者存在两种类型的浆细胞，淀粉样蛋白预计来源于单克隆浆细胞产生的免疫球蛋白轻链。
