Incidence and risk factors for myelofibrotic transformation among 272 chinese patients with JAK2-mutated polycythemia vera

Incidence and risk factors for myelofibrotic transformation among 272 chinese patients with JAK2-mutated polycythemia vera
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272 例中国 JAK2 突变真性红细胞增多症患者骨髓纤维化转化的发生率和危险因素。

DOI:
10.1002/ajh.24191
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发表时间:
2015-12-01
影响因子:
12.8
通讯作者:
Xue, Yanping
Xue, Yanping
中科院分区:
医学1区
文献类型:
--
作者:
Bai, Jie;Ai, Limei;Xue, Yanping

文献摘要

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真性红细胞增多症后骨髓纤维化(PV - MF)是真性红细胞增多症(PV)的关键血液学演变。本研究的主要目的是确定中国PV患者PV后MF发生和预后的可能危险因素。回顾性分析了272例JAK2(V617F)或外显子12突变的中国PV患者。在272例PV患者中,63例发生PV后MF。血小板计数bbb550 × 10(9)/L和脾肿大被确定为pv后MF的独立危险因素。pv后MF患者的中位生存期为8年。贫血和诊断为pv后MF时年龄≥65岁被认为是pv后MF不良预后的重要预测因素。总之,血小板计数和脾大是转化为PV后MF的重要预测因素,而贫血(65岁血红蛋白水平)是JAK2(V617F)或外显子12突变的中国PV患者PV后MF预后不良的重要预测因素。(C) 2015 Wiley期刊公司
Post-polycythemia vera myelofibrosis (post-PV MF) is a critical hematologic evolution of polycythemia vera (PV). The main purpose of the present study was to identify the possible risk factors for the occurrence and prognosis of post-PV MF in Chinese patients with PV. A cohort of 272 Chinese PV patients with JAK2(V617F) or exon12 mutation was retrospectively analyzed. Of the 272 patients with PV, 63 developed post-PV MF. Platelet count >550 x 10(9)/L and splenomegaly were identified as independent risk factors for post-PV MF. The median duration of survival for post-PV MF patients was 8 years. Anemia and age >65 years at diagnosis of post-PV MF were identified as significant predictors for the poor prognosis of post-PV MF. In conclusion, platelet counts and splenomegaly were significant predictors for the transformation to post-PV MF, while anemia (hemoglobin levels 65 years were significant predictors for poor prognosis of post-PV MF in Chinese PV patients with JAK2(V617F) or exon12 mutation. (C) 2015 Wiley Periodicals, Inc.