Monoclonal gammopathies of undetermined significance.

Robert A Kyle, S Vincent Rajkumar
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引用次数: 152

Abstract

The term 'monoclonal gammopathy of undetermined significance' denotes the presence of a monoclonal protein in patients without evidence of multiple myeloma, macroglobulinemia, amyloidosis or related plasma cell proliferative disorders. The disorder has been found in approximately 3% of persons older than 70 years and in approximately 1% of persons older than 50 years. A population-based study included 1384 patients from south-eastern Minnesota who had the disorder diagnosed at the Mayo Clinic from 1960 through 1994. Risk of progression was about 1% per year, but patients were at risk of progression even after 25 years or more of stable monoclonal gammopathy of undetermined significance. The risk for development of multiple myeloma was increased 25-fold; the risk of macroglobulinemia, 46-fold; and the risk of primary amyloidosis, 8.4-fold. Concentration and type of monoclonal protein were the only independent predictors of progression. The presence of a urine monoclonal protein and the reduction of one or more uninvolved immunoglobulins were not risk factors for progression. Monoclonal gammopathy of undetermined significance may be associated with various disorders, including lymphoproliferative diseases, leukemia, von Willebrand disease, connective tissue diseases and neurologic disorders.

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意义不明的单克隆伽玛病。
“意义不明的单克隆伽玛病”是指在没有多发性骨髓瘤、巨球蛋白血症、淀粉样变性或相关浆细胞增生性疾病证据的患者中存在单克隆蛋白。大约3%的70岁以上的老年人和大约1%的50岁以上的老年人发现了这种疾病。一项基于人群的研究包括1384名来自明尼苏达州东南部的患者,这些患者从1960年到1994年在梅奥诊所被诊断出患有这种疾病。病情进展的风险约为每年1%,但患者在患有意义不明的稳定单克隆伽玛病25年或更长时间后仍有进展的风险。发生多发性骨髓瘤的风险增加了25倍;患巨球蛋白血症的风险为46倍;原发性淀粉样变性的风险是8.4倍。单克隆蛋白的浓度和类型是病情进展的唯一独立预测因子。尿单克隆蛋白的存在和一种或多种非相关免疫球蛋白的减少不是进展的危险因素。意义不明的单克隆伽玛病可能与多种疾病有关,包括淋巴细胞增生性疾病、白血病、血管性血友病、结缔组织疾病和神经系统疾病。
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