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意义未明的单克隆丙种球蛋白病预后的长期研究。

A long-term study of prognosis in monoclonal gammopathy of undetermined significance.

作者信息

Kyle Robert A, Therneau Terry M, Rajkumar S Vincent, Offord Janice R, Larson Dirk R, Plevak Matthew F, Melton L Joseph

机构信息

Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.

出版信息

N Engl J Med. 2002 Feb 21;346(8):564-9. doi: 10.1056/NEJMoa01133202.

DOI:10.1056/NEJMoa01133202
PMID:11856795
Abstract

BACKGROUND

A monoclonal gammopathy of undetermined significance (MGUS) occurs in up to 2 percent of persons 50 years of age or older. Reliable predictors of progression have not been identified, and information on prognosis is limited.

METHODS

We identified 1384 patients residing in southeastern Minnesota in whom MGUS was diagnosed at the Mayo Clinic from 1960 through 1994. The primary end point was progression to multiple myeloma or another plasma-cell cancer.

RESULTS

During 11,009 person-years of follow-up, MGUS progressed in 115 of the 1384 patients to multiple myeloma, IgM lymphoma, primary amyloidosis, macroglobulinemia, chronic lymphocytic leukemia, or plasmacytoma (relative risk of progression, 25.0, 2.4, 8.4, 46.0, 0.9, and 8.5, respectively). The overall relative risk of progression was 7.3 in these patients as compared with the white population of the Iowa Surveillance, Epidemiology, and End Results program. In 32 additional patients, the monoclonal protein concentration increased to more than 3 g per deciliter or the percentage of plasma cells in the bone marrow increased to more than 10 percent (smoldering multiple myeloma) but without progression to overt myeloma or related disorders. The cumulative probability of progression was 12 percent at 10 years, 25 percent at 20 years, and 30 percent at 25 years. The initial concentration of serum monoclonal protein was a significant predictor of progression at 20 years.

CONCLUSIONS

The risk of progression of MGUS to multiple myeloma or related disorders is about 1 percent per year.

摘要

背景

意义未明的单克隆丙种球蛋白病(MGUS)在50岁及以上人群中的发生率高达2%。尚未确定可靠的病情进展预测指标,且预后信息有限。

方法

我们确定了1960年至1994年在梅奥诊所被诊断为MGUS的1384名居住在明尼苏达州东南部的患者。主要终点是进展为多发性骨髓瘤或其他浆细胞癌。

结果

在11009人年的随访期间,1384例患者中有115例MGUS进展为多发性骨髓瘤、IgM淋巴瘤、原发性淀粉样变性、巨球蛋白血症、慢性淋巴细胞白血病或浆细胞瘤(进展的相对风险分别为25.0、2.4、8.4、46.0、0.9和8.5)。与爱荷华州监测、流行病学和最终结果项目的白人人群相比,这些患者的总体进展相对风险为7.3。另有32例患者,单克隆蛋白浓度增加至每分升超过3克或骨髓中浆细胞百分比增加至超过10%(冒烟型多发性骨髓瘤),但未进展为明显的骨髓瘤或相关疾病。进展的累积概率在10年时为12%,20年时为25%,25年时为30%。血清单克隆蛋白的初始浓度是20年时进展的重要预测指标。

结论

MGUS进展为多发性骨髓瘤或相关疾病的风险约为每年1%。

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