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意义未明的单克隆丙种球蛋白病中的恶性转化与预期寿命

Malignant transformation and life expectancy in monoclonal gammopathy of undetermined significance.

作者信息

Blade J, Lopez-Guillermo A, Rozman C, Cervantes F, Salgado C, Aguilar J L, Vives-Corrons J L, Montserrat E

机构信息

Postgraduate School of Haematology Farreras-Valentí, Hospital Clínic i Provincial, University of Barcelona, Spain.

出版信息

Br J Haematol. 1992 Jul;81(3):391-4. doi: 10.1111/j.1365-2141.1992.tb08245.x.

DOI:10.1111/j.1365-2141.1992.tb08245.x
PMID:1390212
Abstract

The actuarial probability of malignant transformation and the impact on expected survival were analysed in a series of 128 persons diagnosed with monoclonal gammopathy of undetermined significance (MGUS) over a 20-year period. At a median follow-up of 56 months the M-component remains stable in 101 patients (78.9%), 14 patients (10.9%) have died from non-related disorders and 13 (10.2%) have developed malignant transformation of MGUS (multiple myeloma, 10; primary amyloidosis, two; Waldenström's macroglobulinaemia, one). The actuarial probability of malignant transformation at 5 and 10 years was 8.5% and 19.2%, respectively. When different presenting features were analysed for predictive value of the malignant transformation, the IgA type of MGUS was the only variable associated with a higher probability of such an event (P less than 0.025). Although no significant difference was observed between the survival probability of persons with MGUS and that of the control population, the development of malignant transformation was associated with a shorter survival (P less than 0.001).

摘要

在20年期间,对128例诊断为意义未明的单克隆丙种球蛋白病(MGUS)的患者进行分析,以确定恶变的精算概率及其对预期生存的影响。在中位随访56个月时,101例患者(78.9%)的M蛋白成分保持稳定,14例患者(10.9%)死于无关疾病,13例患者(10.2%)发生MGUS恶变(多发性骨髓瘤10例;原发性淀粉样变性2例;华氏巨球蛋白血症1例)。5年和10年恶变的精算概率分别为8.5%和19.2%。当分析不同临床表现对恶变的预测价值时,IgA类型的MGUS是与该事件较高概率相关的唯一变量(P<0.025)。虽然MGUS患者与对照人群的生存概率无显著差异,但恶变的发生与较短的生存期相关(P<0.001)。

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