Division of Hematology, Department of Internal Medicine, Konkuk University Medical Center, Seoul, Korea.
Department of Internal Medicine, Daegu Catholic University School of Medicine, Daegu, Korea.
Korean J Intern Med. 2021 Jan;36(1):45-62. doi: 10.3904/kjim.2020.319. Epub 2020 Dec 4.
In 2016, the World Health Organization revised the diagnostic criteria for myeloproliferative neoplasms (MPNs) based on the discovery of disease-driving genetic aberrations and extensive analysis of the clinical characteristics of patients with MPNs. Recent studies have suggested that additional somatic mutations have a clinical impact on the prognosis of patients harboring these genetic abnormalities. Treatment strategies have also advanced with the introduction of JAK inhibitors, one of which has been approved for the treatment of patients with myelofibrosis and those with hydroxyurea-resistant or intolerant polycythemia vera. Recently developed drugs aim to elicit hematologic responses, as well as symptomatic and molecular responses, and the response criteria were refined accordingly. Based on these changes, we have revised the guidelines and present the diagnosis, treatment, and risk stratification of MPNs encountered in Korea.
2016 年，世界卫生组织（WHO）根据对驱动性基因异常的发现以及对骨髓增殖性肿瘤（MPN）患者临床特征的广泛分析，对 MPN 的诊断标准进行了修订。最近的研究表明，这些遗传异常患者中存在的其他体细胞突变对预后具有临床影响。随着 JAK 抑制剂的引入，治疗策略也取得了进展，其中一种已被批准用于治疗骨髓纤维化以及对羟基脲耐药或不耐受的真性红细胞增多症患者。最近开发的药物旨在引起血液学反应以及症状和分子反应，相应地对反应标准进行了细化。基于这些变化，我们修订了指南，并呈现了韩国 MPN 的诊断、治疗和风险分层。
