MIPSS70: Mutation-Enhanced International Prognostic Score System for Transplantation-Age Patients With Primary Myelofibrosis
MIPSS70: Mutation-Enhanced International Prognostic Score System for Transplantation-Age Patients With Primary Myelofibrosis
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DOI：
10.1200/jco.2017.76.4886
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发表时间：
2018-02-01
影响因子：
45.3
通讯作者：
Tefferi, Ayalew
中科院分区：
文献类型：
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作者：
Guglielmelli, Paola;Lasho, Terra L.;Tefferi, Ayalew
本研究包括805例移植年龄的原发性骨髓纤维化（PMF）患者，年龄25 * 10（9）/L，血小板< 100 * 10（9）/L，循环原始细胞>= 2%，骨髓纤维化分级> 2，全身症状，不存在CALR 1型突变，存在高分子风险突变（即ASXL 1、EZH 2、SRSF 2、IDH 1/2），以及存在两种或多种高分子风险突变。通过将风险比（HR）加权点分配给这些变量，MIPSS 70模型描绘了三个风险类别;低风险类别的5年OS为95%，中风险类别为70%，高风险类别为29%，对应的中位OS为27.7年（95% CI，22 - 34年）、7.1年（95% CI，6.2 - 8.1年）和2.3年（95% CI，1.9 - 2.7年）。在包括细胞遗传学信息的MIPSS 70-plus模型中，描述了4种风险类别，低风险患者的5年OS为91%，中风险患者为66（HR，3.2; 95% CI，1.9至5.2），高风险类别中为42%（HR，6.4; 95% CI，4.1至10.0），极高风险类别中为7%（HR，17.0; 95% CI，9.8至29.2）。结论MIPSS 70和MIPSS 70-plus为移植年龄PMF患者的风险分层提供了补充系统，并整合了临床、细胞遗传学和突变相关数据。(C)2017年美国临床肿瘤学会
PurposeTo develop a prognostic system for transplantation-age patients with primary myelofibrosis (PMF) that integrates clinical, cytogenetic, and mutation data.Patients and MethodsThe study included 805 patients with PMF age 25 * 10(9)/L, platelets, < 100 * 10(9)/L, circulating blasts >= 2%, bone marrow fibrosis grade > 2, constitutional symptoms, absence of CALR type-1 mutation, presence of high-molecular risk mutation (ie, ASXL1, EZH2, SRSF2, IDH1/2), and presence of two or more high-molecular risk mutations. By assigning hazard ratio (HR)-weighted points to these variables, three risk categories were delineated for the MIPSS70 model; 5-year OS was 95% in low-risk, 70% in intermediate-risk, and 29% in high-risk categories, corresponding to median OS of 27.7 years (95% CI, 22 to 34 years), 7.1 years (95% CI, 6.2 to 8.1 years), and 2.3 years (95% CI, 1.9 to 2.7 years), respectively. In the MIPSS70-plus model, which included cytogenetic information, four risk categories were delineated, with 5-year OS of 91% in low-risk, 66% in intermediate-risk (HR, 3.2; 95% CI, 1.9 to 5.2), 42% in high-risk (HR, 6.4; 95% CI, 4.1 to 10.0), and 7% very high-risk categories (HR, 17.0; 95% CI, 9.8 to 29.2). Both models remained effective after inclusion of older patients in the analysis.ConclusionMIPSS70 and MIPSS70-plus provide complementary systems of risk stratification for transplantation-age patients with PMF and integrate prognostically relevant clinical, cytogenetic, and mutation data. (C) 2017 by American Society of Clinical Oncology