期刊
LEUKEMIA
卷 30, 期 12, 页码 2342-2350出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/leu.2016.190
关键词
-
资金
- Deutsche Jose Carreras Leukamie-Stiftung e.V. [DJCLS H 11/03, R 13/05]
- Austrian Science Fund (FWF) [SFB F4704-B20]
We evaluated the impact of clinical and molecular characteristics on overall survival (OS) in 108 patients with indolent (n = 41) and advanced systemic mastocytosis (SM) (advSM, n = 67). Organomegaly was measured by magnetic resonance imaging-based volumetry of the liver and spleen. In multivariate analysis of all patients, an increased spleen volume >= 450 ml (hazard ratio (HR), 5.2; 95% confidence interval (CI), (2.1-13.0); P = 0.003) and an elevated alkaline phosphatase (AP; HR 5.0 (1.1-22.2); P = 0.02) were associated with adverse OS. The 3-year OS was 100, 77, and 39%, respectively (P<0.0001), for patients with 0 (low risk, n = 37), 1 (intermediate risk, n = 32) or 2 (high risk, n = 39) parameters. For advSM patients with fully available clinical and molecular data (n = 60), univariate analysis identified splenomegaly. 1200 ml, elevated AP and mutations in the SRSF2/ASXL1/RUNX1 (S/A/R) gene panel as significant prognostic markers. In multivariate analysis, mutations in S/A/R (HR 3.2 (1.1-9.6); P = 0.01) and elevated AP (HR 2.6 (1.0-7.1); P = 0.03) remained predictive adverse prognostic markers for OS. The 3-year OS was 76 and 38%, respectively (P = 0.0003), for patients with 0-1 (intermediate risk, n = 28) or 2 (high risk, n = 32) parameters. We conclude that splenomegaly, elevated AP and mutations in the S/A/R gene panel are independent of the World Health Organization classification and provide the most relevant prognostic information in SM patients.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据