原发性血小板增多症
医学
钙网蛋白
骨髓纤维化
真性红细胞增多症
内科学
疾病
血液学
基因突变
肿瘤科
基因
突变
骨髓
遗传学
生物
内质网
出处
期刊:PubMed
日期:2022-01-01
卷期号:63 (9): 1083-1091
标识
DOI:10.11406/rinketsu.63.1083
摘要
Since the discovery of the JAK2V617F, MPL gene, and Calreticulin gene mutations, remarkable changes have occurred in the identification of the pathology of Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). The diagnostic criteria of polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the world health organization classification systems have also been amended to include these driver gene mutations. Additionally, treatment algorithms for each disease have been reviewed. Following these changes, real world data form several countries based on national surveys have been reported. In Japan, the Japanese Society of Hematology has conducted a prospective study, named the JSH-MPN-15 study, to investigate the overall survival and risk factors of patients with MPNs. Furthermore, the retrospective JSH-MPN-R18 study was conducted and the results have been coming out. In this lecture, the results of these studies will be discussed.
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