Thalassemia & Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences , Ahvaz, Iran.
Student Research Committee, Ahvaz Jundishapur University of Medical Sciences , Ahvaz, Iran.
Expert Rev Hematol. 2020 Nov;13(11):1289-1301. doi: 10.1080/17474086.2020.1819232. Epub 2020 Sep 29.
Myeloproliferative neoplasms (MPNs) are a group of clonal hematopoietic stem cell disorders that may occur after one or more mutations in hematopoietic progenitor cells. In this study, we will review the co-existence of mutations (especially dual mutations) in MPNs and its effect on the prognosis of patients.
To find relevant published papers, we systematically searched six major international indexing databases, namely PubMed/Medline, EmBase, Cochrane central, ISI web of science, and Scopus from Feb. 2000 until Jan. 2020. We included the following keywords in the analyzes: Myeloproliferative Disorders, Mutation, Co-existence of Mutations, Acute myeloid leukemia.
Co-existence of several mutations in MPNs is mainly associated with a poor prognosis compared with the unimutated MPN disorders. There are several effective factors such as sequence of mutations, incidence of mutations in one cell or different cells, mutation, and MPN type.
It seems that monitoring the status of mutations in MPNs and recognizing the co-existence of mutations (especially dual mutations) in order to determine prognosis and possibility of progression to acute form of leukemia can lead to the prediction of prognosis in MPN patients as well as establishment of better and more reliable therapeutic strategies for patients.
骨髓增殖性肿瘤（MPN）是一组造血干细胞克隆性疾病，可能发生于造血祖细胞的一次或多次突变后。本研究将综述 MPN 中突变（尤其是双重突变）的共存及其对患者预后的影响。
为了寻找相关的已发表文献，我们系统地检索了六个主要的国际索引数据库，即 PubMed/Medline、Embase、Cochrane 中心、ISI web of science 和 Scopus，检索时间为 2000 年 2 月至 2020 年 1 月。我们在分析中使用了以下关键词：骨髓增殖性疾病、突变、突变共存、急性髓系白血病。
MPN 中几种突变的共存与未突变的 MPN 疾病相比，主要与预后不良相关。有几个有效的因素，如突变的顺序、一个细胞或不同细胞中突变的发生率、突变和 MPN 类型。
似乎监测 MPN 中的突变状态，并识别突变的共存（尤其是双重突变），以确定预后和向急性白血病形式进展的可能性，可以预测 MPN 患者的预后，并为患者建立更好、更可靠的治疗策略。
