State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, People's Republic of China.
Department of Hematology, The First Affiliated Hospital of Liaoning Medical University, Jinzhou, People's Republic of China.
Am J Hematol. 2015 Dec;90(12):1116-21. doi: 10.1002/ajh.24191. Epub 2015 Nov 17.
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 × 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 <100 g/L) and age>65 years were significant predictors for poor prognosis of post-PV MF in Chinese PV patients with JAK2(V617F) or exon12 mutation.
原发性骨髓纤维化后(post-PV MF)是原发性骨髓纤维化(PV)的一个重要血液学演变。本研究的主要目的是确定中国 PV 患者发生 post-PV MF 的可能危险因素及其预后。回顾性分析了 272 例 JAK2(V617F)或外显子 12 突变的中国 PV 患者队列。在 272 例 PV 患者中,有 63 例发生 post-PV MF。血小板计数>550×10(9)/L 和脾肿大被确定为 post-PV MF 的独立危险因素。post-PV MF 患者的中位生存时间为 8 年。贫血和诊断后 post-PV MF 年龄>65 岁被确定为 post-PV MF 不良预后的显著预测因素。总之,血小板计数和脾肿大是向 post-PV MF 转化的显著预测因素,而贫血(血红蛋白水平<100g/L)和诊断后 post-PV MF 年龄>65 岁是中国 JAK2(V617F)或外显子 12 突变的 PV 患者 post-PV MF 不良预后的显著预测因素。