Monoclonal gammopathies of undetermined significance.
Monoclonal gammopathies of undetermined significance.
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DOI：
10.1046/j.1468-0734.2002.00076.x
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发表时间：
2002-09-01
期刊：
影响因子：
--
通讯作者：
Rajkumar, S Vincent
中科院分区：
文献类型：
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作者：
Kyle, Robert A;Rajkumar, S Vincent
术语“意义未定的单克隆丙种球蛋白病”是指患者体内存在单克隆蛋白，但没有多发性骨髓瘤、巨球蛋白血症、淀粉样变性或相关浆细胞增殖性疾病的证据。大约 3% 的 70 岁以上人群和大约 1% 的 50 岁以上人群中发现了这种疾病。一项以人群为基础的研究纳入了来自明尼苏达州东南部的 1384 名患者，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。疾病进展的风险约为每年 1%，但即使在意义未确定的稳定单克隆丙种球蛋白病持续 25 年或更长时间后，患者仍有疾病进展的风险。患多发性骨髓瘤的风险增加 25 倍；巨球蛋白血症的风险增加 46 倍；原发性淀粉样变性的风险是原来的 8.4 倍。单克隆蛋白的浓度和类型是进展的唯一独立预测因子。尿液单克隆蛋白的存在和一种或多种不受影响的免疫球蛋白的减少不是进展的危险因素。意义未明的单克隆丙种球蛋白病可能与多种疾病相关，包括淋巴增殖性疾病、白血病、冯维勒布兰德病、结缔组织疾病和神经系统疾病。
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.