文章：

骨髓纤维化治疗流程图（2018年版）

Myelofibrosis Treatment Algorithm 2018

原文发布日期：2018-07-31

DOI: 10.1038/s41408-018-0109-0

类型: Current Treatment Algorithm

开放获取: 是

英文摘要：

Two novel prognostic systems for primary myelofibrosis (PMF) were recently unveiled: GIPSS (genetically inspired prognostic scoring system) and MIPSS70 (mutation-enhanced international prognostic scoring system for transplant-age patients). GIPSS is based exclusively on genetic markers: mutations and karyotype. MIPSS70 includes mutations and clinical risk factors. In its most recent adaptation, the prognostic value of MIPSS70 has been bolstered by the inclusion of a three-tiered cytogenetic risk stratification and use of hemoglobin thresholds that are adjusted for sex and severity (MIPSS70+ version 2.0). GIPSS features four, MIPSS70 three, and MIPSS70+ version 2.0 five risk categories. MIPSS70 is most useful in the absence of cytogenetic information. MIPSS70+ version 2.0 is more comprehensive than MIPSS70 and is the preferred model in the presence of cytogenetic information. Both MIPSS70 and MIPSS70+ version 2.0 require an online score calculator (http://www.mipss70score.it). GIPPS offers a lower complexity prognostic tool that reliably identifies candidates for allogeneic stem cell transplant (GIPSS high-risk disease) or long-term observation with little or no therapeutic intervention (GIPSS low-risk disease). Ultimately, we favor a step-wise prognostication approach that starts with GIPSS but also considers MIPSS70+ version 2.0 for confirming the most appropriate treatment approach for the individual patient.

摘要翻译： 

最近发布了两种针对原发性骨髓纤维化（PMF）的新型预后系统：GIPSS（遗传启发预后评分系统）和MIPSS70（针对移植适龄患者的突变增强国际预后评分系统）。GIPSS完全基于遗传标志：突变和核型。MIPSS70则包含突变和临床风险因素。在其最新版本中，MIPSS70通过纳入三级细胞遗传学风险分层以及采用根据性别和严重程度调整的血红蛋白阈值（MIPSS70+ 2.0版），增强了预后评估价值。GIPSS包含四个风险类别，MIPSS70包含三个，MIPSS70+ 2.0版则包含五个风险类别。在缺乏细胞遗传学信息时，MIPSS70最为实用。MIPSS70+ 2.0版比MIPSS70更全面，是在有细胞遗传学信息时的首选模型。MIPSS70和MIPSS70+ 2.0版均需要在线评分计算器（http://www.mipss70score.it）进行操作。GIPSS提供了一个复杂度较低的预后工具，能可靠识别适合进行异基因干细胞移植的候选者（GIPSS高风险疾病）或适合长期观察且无需或仅需极少治疗干预的候选者（GIPSS低风险疾病）。最终，我们倾向于采用阶梯式预后评估方法：从GIPSS开始，同时结合MIPSS70+ 2.0版来确认最适合个体患者的治疗方案。

原文链接：

Myelofibrosis Treatment Algorithm 2018