意义未明单克隆免疫球蛋白血症轻链型的流行率和进展风险：一项回顾性基于人群的队列研究。
Prevalence and risk of progression of light-chain monoclonal gammopathy of undetermined significance: a retrospective population-based cohort study.
机构信息
Hematology, Mayo Clinic, Rochester, MN 55905, USA.
出版信息
Lancet. 2010 May 15;375(9727):1721-8. doi: 10.1016/S0140-6736(10)60482-5.
BACKGROUND
Monoclonal gammopathy of undetermined significance (MGUS) is defined by expression of heavy-chain immunoglobulin (IgH) and is the precursor lesion for 80% of cases of multiple myeloma. The remaining 20% are characterised by absence of IgH expression; we aimed to assess prevalence of a corresponding precursor entity, light-chain MGUS.
METHODS
We used a population-based cohort, previously assembled to estimate MGUS prevalence, of 21,463 residents of Olmsted County, MN, USA, aged 50 years and older. We did a serum free light-chain assay on all samples with sufficient serum remaining, and immunofixation electrophoresis was done for all samples with an abnormal free light-chain ratio or abnormal protein electrophoresis results from the original study. Light-chain MGUS was defined as an abnormal free light-chain ratio with no IgH expression, plus increased concentration of the involved light chain. We calculated age-specific and sex-specific prevalence and rates of progression to lymphoproliferative disorders for light-chain and conventional MGUS and assessed incidence of renal disorders in patients with light-chain MGUS.
FINDINGS
610 (3.3%) of 18,357 people tested had an abnormal free light-chain ratio, of whom 213 had IgH expression that was diagnostic of conventional MGUS. 146 of the remaining 397 individuals had an increase of at least one free light chain and met criteria for light-chain MGUS. Prevalence of light-chain MGUS was 0.8% (95% CI 0.7-0.9), contributing to an overall MGUS prevalence of 4.2% (3.9-4.5). Risk of progression to multiple myeloma in patients with light-chain MGUS was 0.3% (0.1-0.8) per 100 person-years. 30 (23%) of 129 patients with light-chain MGUS were diagnosed with renal disease.
INTERPRETATION
We define a clinical entity representing the light-chain equivalent of conventional MGUS and posing a risk of progression to light-chain multiple myeloma and related disorders.
FUNDING
US National Cancer Institute.
背景
意义未明的单克隆丙种球蛋白血症（MGUS）由重链免疫球蛋白（IgH）表达定义，是 80%多发性骨髓瘤病例的前体病变。其余 20%的病例特征是缺乏 IgH 表达；我们旨在评估相应的前体实体，即轻链 MGUS 的患病率。
方法
我们使用了一个基于人群的队列，该队列以前是为了估计明尼苏达州奥姆斯特德县 50 岁及以上的 21463 名居民的 MGUS 患病率而组建的。我们对所有剩余血清充足的样本进行血清游离轻链检测，并对所有游离轻链比值异常或原始研究中蛋白电泳结果异常的样本进行免疫固定电泳。轻链 MGUS 的定义为异常游离轻链比值且无 IgH 表达，同时涉及的轻链浓度增加。我们计算了轻链和常规 MGUS 的年龄和性别特异性患病率和进展为淋巴增生性疾病的比率，并评估了轻链 MGUS 患者的肾脏疾病发生率。
结果
在 18357 名接受检测的人中，有 610 人（3.3%）的游离轻链比值异常，其中 213 人有 IgH 表达，诊断为常规 MGUS。在其余 397 人中，有 146 人的至少一条游离轻链增加且符合轻链 MGUS 的标准。轻链 MGUS 的患病率为 0.8%（95%CI 0.7-0.9），总体 MGUS 患病率为 4.2%（3.9-4.5）。轻链 MGUS 患者进展为多发性骨髓瘤的风险为每 100 人年 0.3%（0.1-0.8）。在 129 名轻链 MGUS 患者中，有 30 名（23%）被诊断为肾脏疾病。
结论
我们定义了一种代表常规 MGUS 的轻链等效物的临床实体，其存在进展为轻链多发性骨髓瘤和相关疾病的风险。
资助
美国国家癌症研究所。
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