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预测骨髓纤维化患者接受低强度移植后生存的风险模型。

Risk models predicting survival after reduced-intensity transplantation for myelofibrosis.

机构信息

Department of Stem Cell Transplantation, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany.

出版信息

Br J Haematol. 2012 Apr;157(1):75-85. doi: 10.1111/j.1365-2141.2011.09009.x. Epub 2012 Jan 27.

DOI:10.1111/j.1365-2141.2011.09009.x
PMID:22280409
Abstract

To define a prognostic model for predicting outcome of reduced-intensity allogeneic stem cell transplantation (RIC-ASCT) for myelofibrosis we evaluated 150 homogeneously treated patients and developed a new risk score for overall survival (OS). In a multivariate Cox model for OS, only JAK2 V617F wild-type, age ≥57 years and constitutional symptoms were independently predictive for OS (Hazard Ratio: 2·02; 2·43 and 2·80 respectively). Depending on the presence of one, two or all of these factors, HR of death was 3·08; 4·70 and 16·61 respectively (P < 0·001). This score was compared to the Lille, Cervantes, International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS) and modified European Blood and Marrow Transplantation Group (EBMT) scores. Lille score correlated significantly with OS but discriminated poorly between the intermediate and high-risk groups (5-year OS 56% and 51% respectively). IPSS and DIPSS correlated significantly with OS but differences between intermediate-1 and intermediate-2 groups were not significant (5-year OS 78% vs. 78% and 70%, 60% respectively). Modified EBMT and Cervantes models did not predict OS post-ASCT. In conclusion, a simple model which includes: age, JAK2 V617F-status and constitutional symptoms can clearly separate distinct risk groups and can be used in addition to the Lille model to predict OS after RIC-ASCT for myelofibrosis.

摘要

为了定义一个预测低强度异基因造血干细胞移植(RIC-ASCT)治疗骨髓纤维化结局的预后模型,我们评估了 150 例同质治疗的患者,并制定了新的总生存(OS)风险评分。在 OS 的多变量 Cox 模型中,仅 JAK2 V617F 野生型、年龄≥57 岁和全身症状是独立预测 OS 的因素(危险比:2.02;2.43 和 2.80)。根据这些因素的存在情况(1、2 或 3 个),死亡的 HR 分别为 3.08、4.70 和 16.61(P<0.001)。该评分与 Lille、Cervantes、国际预后评分系统(IPSS)、动态 IPSS(DIPSS)和改良欧洲血液和骨髓移植组(EBMT)评分进行了比较。Lille 评分与 OS 显著相关,但在中危和高危组之间的区分较差(5 年 OS 分别为 56%和 51%)。IPSS 和 DIPSS 与 OS 显著相关,但中危-1 组和中危-2 组之间的差异无统计学意义(5 年 OS 分别为 78%、78%和 70%、60%)。改良 EBMT 和 Cervantes 模型不能预测 ASCT 后 OS。总之,一个包含年龄、JAK2 V617F 状态和全身症状的简单模型可以明确区分不同的风险组,并可与 Lille 模型一起用于预测骨髓纤维化 RIC-ASCT 后的 OS。

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