骨髓增殖性疾病的研究进展：后JAK2突变时代的几个临床问题

Research progress of myeloproliferative disorders： several clinical problems post the JAK2 mutation era

系统复习了骨髓增殖性疾病（MPD）研究领域JAK2V617F突变的相关进展，认同JAK2V617F突变的发现在MPD研究中具有重要意义。着重讨论了后JAK2突变时代几个敏感的问题，表达了初步看法：接受世界卫生组织（WHO）2008年推荐的对MPD诊断标准的建议；对MPD／骨髓增殖性肿瘤（myeloproliferative neoplasm, MPN）的命名有不同的解读，建议采用MPD，而不采纳MPN的称谓；提出了对MPD（不含CML）治疗的建议：在有效的靶向JAK2V617F抑制剂应用于MPD治疗之前，针对其骨髓过度增殖可短期使用羟基脲（HU），推荐同时较长疗程应用干扰素-α（IFN-α），及时采用低剂量阿司匹林，以预防血栓形成等并发症。

This critical review was summarized more systematically about the JAK2V617F mutation of related research progress in myeloproliferative disorders （MPD） research fields and the identification of JAK2V617F mutation represents an important advance in our understanding of MPD was agreed. The authors focused on several sensitive problems of post the JAK2 mutation era, and expressed their opinions. The Guideline of the MPD diagnostic criteria recommended by WHO in 2008 was accepted. The authors recommend the MPD, rather than myeloproliferative neoplasm （MPN）. The treatment for the MPD （not including the CML） is recommended. Before the effective targeting of JAK2V617F specific inhibitors for the treatment of the MPD, short-term of use hydroxyurea （HU） was suggested to suppress excessive proliferation of bone marrow of MPD and a long course of treatment application of inteferon-α（IFN-α）, and low-dose of aspirin in a timely manner were recommended to prevent thrombosis and other complications.