Risk stratification based on both disease status and extra-hematologic comorbidities in patients with myelodysplastic syndrome
Risk stratification based on both disease status and extra-hematologic comorbidities in patients with myelodysplastic syndrome
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DOI：
10.3324/haematol.2010.033506
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发表时间：
2011-03-01
期刊：
影响因子：
--
通讯作者：
Cazzola, Mario
中科院分区：
文献类型：
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作者：
Della Porta, Matteo G.;Malcovati, Luca;Cazzola, Mario
骨髓增生异常综合征的发病率随着年龄的增长而增加，并且在这些患者中报告了高患病率的共病状况。到目前为止，骨髓增生异常综合征的风险评估主要基于疾病状态。我们研究了合并症对骨髓增生异常综合征自然史的预后影响，目的是开发新的风险评估工具。研究人群包括在意大利帕维亚诊断为骨髓增生异常综合征的840例患者的学习队列和在德国杜塞尔多夫随访的504例患者的验证队列。合并症的信息是从诊断时和病程中对患者病历和实验室检查值的详细审查中提取的。使用考克斯比例风险回归模型进行单变量和多变量生存分析，包括固定和时间依赖性协变量。学习队列中54%的患者存在合并症。心脏病是最常见的合并症和非白血病死亡的主要原因。在多变量分析中，合并症对非白血病死亡（P=0.01）和总生存率（P=0.02）有显著影响。心脏、肝脏、肾脏、肺部疾病和实体瘤被发现独立影响非白血病死亡的风险。建立了时间依赖性骨髓增生异常综合征特异性合并症指数（MDS-CI），用于预测合并症对预后的影响。这确定了三组患者，其在验证队列中也显示出显著不同的非白血病死亡（P < 0.001）和存活（P=0.005）概率。在确诊后的固定时间点进行的标志性生存分析显示，MDS-CI能够更好地定义根据WHO分类的预后评分系统（WPSS）分层的骨髓增生异常综合征患者的预期寿命。通过WPSS和MDS-CI对疾病状态和合并症进行解释，大大改善了骨髓增生异常综合征的风险分层。
The incidence of myelodysplastic syndromes increases with age and a high prevalence of co-morbid conditions has been reported in these patients. So far, risk assessment in myelodysplastic syndromes has been mainly based on disease status. We studied the prognostic impact of comorbidity on the natural history of myelodysplastic syndrome with the aim of developing novel tools for risk assessment. The study population included a learning cohort of 840 patients diagnosed with myelodysplastic syndrome in Pavia, Italy, and a validation cohort of 504 patients followed in Duesseldorf, Germany. Information on comorbidity was extracted from detailed review of the patients medical charts and laboratory values at diagnosis and during the course of the disease. Univariable and multivariable survival analyses with both fixed and time-dependent covariates were performed using Cox's proportional hazards regression models. Comorbidity was present in 54% of patients in the learning cohort. Cardiac disease was the most frequent comorbidity and the main cause of non-leukemic death. In multivariable analysis, comorbidity had a significant impact on both non-leukemic death (P=0.01) and overall survival (P=0.02). Cardiac, liver, renal, pulmonary disease and solid tumors were found to independently affect the risk of non-leukemic death. A time-dependent myelodysplastic syndrome-specific c:omorbidity index (MDS-CI) was developed for predicting the effect of comorbidity on outcome. This identified three groups of patients which showed significantly different probabilities of non-leukemic death (P < 0.001) and survival (P=0.005) also in the validation cohort. Landmark survival analyses at fixed time points from diagnosis showed that the MDS-CI can better define the life expectancy of patients with myelodysplastic syndrome stratified according to the WHO-classification based Prognostic Scoring System (WPSS).Comorbidities have a significant impact on the outcome of patients with myelodysplastic syndrome. Accounting for both disease status by means of the WPSS and comorbidity through the MDS-CI considerably improves risk stratification in myelodysplastic syndromes.