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原发性AL淀粉样变性患者血清游离轻链水平与骨髓浆细胞表型的相关性

Correlation between serum levels of free light chain and phenotype of plasma cells in bone marrow in primary AL amyloidosis.

作者信息

Shimojima Yasuhiro, Matsuda Masayuki, Gono Takahisa, Ishii Wataru, Fushimi Tomohisa, Hoshii Yoshinobu, Yamada Toshiyuki, Ikeda Shu-Ichi

机构信息

Third Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan.

出版信息

Amyloid. 2005 Mar;12(1):33-40. doi: 10.1080/02772240500032515.

DOI:10.1080/02772240500032515
PMID:16076609
Abstract

To investigate whether there is a correlation between subtypes of plasma cells in the bone marrow and the production of M-protein, flow cytometry and serum free light chain (FLC) analyses were carried out in 17 patients with primary systemic AL amyloidosis (mean age, 59.9+/-8.8 years) and controls with M-protein (MGUS controls, n=6) and without it (negative controls, n=9). The patients showed a significantly higher value in the serum predominant FLC:serum creatinine ratio (43.8+/-63.2) and CD38++ CD19- CD56+ subpopulation (monoclonal plasma cells) (2.57+/-5.35%) than either the negative (p<0.0005 and p<0.001, respectively) or MGUS controls (p<0.05). With respect to maturation of plasma cells in the bone marrow, the intermediate (MPC-1+ CD45- CD49e-) and mature (MPC-1+ CD45+ CD49e-) subtypes were significantly higher (49.2+/-23.2%, p<0.005) and lower (27.6+/-21.3%, p<0.005) in the patients than in the negative controls, respectively. The serum predominant FLC:serum creatinine ratio was elevated in parallel with an increase in CD38++ CD19- CD56+ and MPC-1+ CD45- CD49e- cells and a decrease in mature subtypes (MPC-1+ CD45+ CD49e- and MPC-1+ CD45+ CD49e+ cells), There was a significantly positive correlation between the serum predominant FLC:serum creatinine ratio and either CD38++ CD19- CD56+ (r=0.510, p<0.05) or MPC-1+ CD45- CD49e- cells (r=0.481, p<0.05). In primary AL amyloidosis M-protein is probably produced by increased monoclonal plasma cells in the bone marrow, particularly by the intermediate subpopulation with a phenotype of MPC-1+ CD45- CD49e-.

摘要

为了研究骨髓中浆细胞亚型与M蛋白产生之间是否存在相关性,对17例原发性系统性AL淀粉样变性患者(平均年龄59.9±8.8岁)以及伴有M蛋白的对照者(MGUS对照,n = 6)和不伴有M蛋白的对照者(阴性对照,n = 9)进行了流式细胞术和血清游离轻链(FLC)分析。患者血清中优势FLC与血清肌酐比值(43.8±63.2)以及CD38++ CD19- CD56+亚群(单克隆浆细胞)(2.57±5.35%)的值显著高于阴性对照(分别为p<0.0005和p<0.001)或MGUS对照(p<0.05)。关于骨髓中浆细胞的成熟情况,与阴性对照相比,患者的中间型(MPC-1+ CD45- CD49e-)和成熟型(MPC-1+ CD45+ CD49e-)亚型分别显著升高(49.2±23.2%,p<0.005)和降低(27.6±21.3%,p<0.005)。血清中优势FLC与血清肌酐比值随着CD38++ CD19- CD56+和MPC-1+ CD45- CD49e-细胞的增加以及成熟亚型(MPC-1+ CD45+ CD49e-和MPC-1+ CD45+ CD49e+细胞)的减少而升高。血清中优势FLC与血清肌酐比值与CD38++ CD19- CD56+(r = 0.510,p<0.05)或MPC-1+ CD45- CD49e-细胞(r = 0.481,p<0.05)之间存在显著正相关。在原发性AL淀粉样变性中,M蛋白可能由骨髓中增加的单克隆浆细胞产生,特别是具有MPC-1+ CD45- CD49e-表型的中间亚群。

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