Clinical characteristics of Japanese patients with polycythemia vera: results of the JSH-MPN-R18 study
Clinical characteristics of Japanese patients with polycythemia vera: results of the JSH-MPN-R18 study
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日本真性红细胞增多症患者的临床特征：JSH-MPN-R18 研究结果
DOI：
10.1007/s12185-022-03412-x
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发表时间：
2022
影响因子：
2.1
通讯作者：
et al.
中科院分区：
文献类型：
--
作者：
Edahiro Yoko;Tomita Akihiro;et al.
存在JAK 2 V617 F或JAK 2外显子12突变是2017年世界卫生组织分类中列出的诊断真性红细胞增多症（PV）的三大标准之一。然而，自从JAK 2突变被发现以来，日本还没有进行过全国性的研究。因此，日本血液学会（JSH）回顾性分析了2005年4月至2018年3月期间诊断的596例日本PV患者的临床特征。在473例有完整JAK 2突变数据的患者中，446例（94.3%）JAK 2 V617 F和10例（2.1%）JAK 2外显子12突变阳性。在中位随访46个月（范围：0-179个月）期间，发生47例（7.9%）死亡。主要死因为继发性恶性肿瘤（23.4%）、急性白血病（12.8%）、非白血病进展性疾病（10.6%）、血栓性（6.4%）和出血性并发症（6.4%）。在临床过程中，分别有4.0%（n= 24）和3.5%（n= 21）的患者发生血栓形成和出血事件。这些结果表明，国际PV预后评分（年龄、静脉血栓形成和白细胞增多）适用于日本PV患者。
The presence of aJAK2V617F orJAK2exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery ofJAK2mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data onJAK2mutations available, 446 (94.3%) and 10 (2.1%) were positive for theJAK2V617F andJAK2exon 12 mutations, respectively. During a median follow-up of 46 months (range: 0–179 months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n= 24) and 3.5% (n= 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV.