天津医药 ›› 2018, Vol. 46 ›› Issue (8): 804-807.doi: 10.11958/20181021

• 专题-专家论坛 • 上一篇    下一篇

骨髓增生异常综合征预后评分

常春康, 许峰   

  1. 上海交通大学附属第六人民医院血液内科 (邮编200233)
  • 收稿日期:2018-07-03 修回日期:2018-07-09 出版日期:2018-08-15 发布日期:2018-08-23
  • 通讯作者: 常春康 E-mail:changchunkang7010@aliyun.com

Prognostic scoring of myelodysplastic syndromes

CHANG Chun-kang, XU Feng   

  1. Department of Hematology, Shanghai Jiaotong University Affiliated Sixth People’ s Hospital, Shanghai 200233, China
  • Received:2018-07-03 Revised:2018-07-09 Published:2018-08-15 Online:2018-08-23

摘要: 骨髓增生异常综合征 (MDS) 是一组起源于造血干/祖细胞的克隆性疾病, 呈现高度的异质性和复杂性。精确的预后评估是本病的一个核心难题, 主要因为该病复杂的发病机制 (基因组、 表观遗传、 骨髓微环境和免疫因素)决定了临床特征的异质性 (年龄、 感染、 出血风险及合并症) 和差异化的临床转归 (骨髓衰竭和白血病转化)。因此,如何综合考量各种因素建立预后积分系统来预测MDS患者的预后和临床转归非常重要。在过去的二十年中, 许多学者致力于研究各种MDS预后评分系统, 典型的预后评分系统例如国际预后评分系统 (IPSS) 及其修订版 (IPSS-R)已被广泛应用。除此之外, 随着众多具有潜在预后预测价值的临床标志和分子学改变的发现, 基于这些新的预后因素的预后评分系统也展现了良好的预后价值。本文总结了近几年MDS的预后评分系统以供临床实践借鉴。

关键词: 骨髓增生异常综合征, 预后, 评分, 国际预后评分系统

Abstract: Myelodysplastic syndrome (MDS) is a clonal disease derived from the hematopoietic stem cells, with a high degree of heterogeneity and complexity. The prognostic evaluation of MDS is a core problem of this disease, mainly because the complex pathogenesis (genomics, epigenetics, bone marrow microenvironment and immune factors) determines the heterogeneity of clinical characteristics (heterogeneity of age, infection, risk of bleeding, and comorbidities) and differential clinical outcome (bone marrow failure and leukemia transformation). Therefore, how to comprehensively consider various prognostic factors to establish a prognostic score system to predict the prognosis and clinical outcome of patients with MDS is very important. In the past two decades, many MDS scholars have devoted themselves to the study of various MDS prognostic scoring systems. Typical prognostic scoring systems such as the International Prognostic Scoring System (IPSS) and its revisions (IPSS-R) have been widely used. In addition, based on these new prognostic factors, the prognostic scoring system has also showed good prognostic value with the discovery of many clinical markers and molecular changes. This review summarizes the prognostic scoring system and new prognostic factors of MDS in recent years for reference in clinical practice.

Key words: myelodysplastic syndrome, prognostic, scoring, the international prognostic scoring system