Prevalence and risk of progression of light-chain monoclonal gammopathy of undetermined significance: a retrospective population-based cohort study.

Prevalence and risk of progression of light-chain monoclonal gammopathy of undetermined significance: a retrospective population-based cohort study.
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DOI:
10.1016/s0140-6736(10)60482-5
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发表时间:
2010-05-15
期刊:
影响因子:
168.9
通讯作者:
Rajkumar, S. Vincent
Rajkumar, S. Vincent
中科院分区:
医学1区
文献类型:
--
作者:
Dispenzieri, Angela;Katzmann, Jerry A.;Kyle, Robert A.;Larson, Dirk R.;Melton, L. Joseph, III;Colby, Colin L.;Therneau, Terry M.;Clark, Raynell;Kumar, Shaji K.;Bradwell, Arthur;Fonseca, Rafael;Jelinek, D. F.;Rajkumar, S. Vincent

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意义不明的单克隆丙种球蛋白病(MGUS)是一种常见的癌前浆细胞疾病,其定义为免疫球蛋白重链(IgH表达)的证据。MGUS是大多数多发性骨髓瘤(MM)病例的前驱病变,但高达20%的MM的特征是完全缺乏IgH表达;相应的前驱实体轻链MGUS(LC-MGUS)的患病率尚不清楚。我们研究了明尼苏达州奥姆斯特德县18,357名年龄在50岁或以上的居民的血清游离轻链(FLC)测定。LC-MGUS定义为完全缺乏IgH表达的异常FLC-R,加上适当受累FLC的升高。3.3%的受试者存在FLC-R异常。其中,213例在常规MGUS的免疫固定诊断上具有IgH表达,其中57例先前未检测到。在其余397名患者中,146名患者至少有一个FLC升高,符合LC-MGUS标准。LC-MGUS的患病率为0.8%,使50岁及以上人群中MGUS+LC-MGUS的总体患病率达到4.2%。LC-MGUS患者进展为MM的风险为0.3%(95%置信区间[CI],0.1 - 0.8%)/年。23%的LC-MGUS患者在获得测试样本时或之后患有肾脏疾病。LC-MGUS在约1%的主要为高加索人的一般人群中流行,其进展为轻链MM和相关疾病的风险与低风险MGUS相当。LC-MGUS也与肾脏疾病风险相关。
Monoclonal gammopathy of undetermined significance (MGUS) is a common premalignant plasma cell disorder defined by evidence of immunoglobulin heavy chain (IgH expression). MGUS is the precursor lesion for most cases of multiple myeloma (MM), but up to 20% of MM is characterized by complete lack of IgH expression; the prevalence of a corresponding precursor entity, light chain MGUS (LC-MGUS) is unknown. We studied 18,357 Olmsted County, Minnesota, residents age 50 years or older with the serum free light chain (FLC) assay. LC-MGUS was defined as an abnormal FLC-R with complete lack of IgH expression, plus elevation in the appropriate involved FLC. An abnormal FLC-R was observed in 3.3% persons tested. Of these, 213 had IgH expression on immunofixation diagnostic of conventional MGUS, 57 of whom were previously undetected. Among the remaining 397 individuals, 146 had elevation of at least one FLC and met criteria for LC-MGUS. The prevalence of LC-MGUS was 0.8%, bringing the overall prevalence of MGUS+LC-MGUS to 4.2% among persons age 50-years and older. The risk of progression to MM among the LC-MGUS patients was 0.3% (95% confidence interval [CI], 0.1 to 0.8%)/year. Twenty-three percent of LC-MGUS patients had renal disease either at the time of or subsequent to acquisition of the test sample. LC-MGUS is prevalent in approximately 1% of a predominantly Caucasian, general population and poses a risk of progression to light-chain MM and related conditions comparable to that of low-risk MGUS. LC-MGUS was also associated with a risk for renal disease.