Abstract

目的评估适用于原发性骨髓化(PMF)患者的国际预后积分系统(IPSS)、动态国际预后积分系统(DIPSS)、修订的动态国际预后积分系统(DIPSS plus)、基于我国PMF患者临床特征提出的修订IPSS(IPSS-Chinese)、修订DIPSS(DIPSS-Chinese)及针对欧美真性红细胞增多症(PV)后骨髓纤维化(post-PV MF)和原发性血小板增多症(ET)后骨髓纤维化(post-ET MF)患者制定的继发性骨髓纤维化预后模型(MYSEC-PM)对我国post-PV MF和post-ET MF患者的预后效力,并探讨post-PV/ET MF患者的预后因素。方法回顾性分析1984年3月至2013年12月期间连续收治的55例post-PV/ET MF患者的临床资料,使用IPSS、DIPSS、DIPSS plus、IPSS-Chinese、DIPSS-Chinese和MYSEC-PM对患者进行预后分组及评估,并对可能的预后因素进行分析。结果55例post-PV/ET MF患者中男20例、女35例,中位年龄59(20~88)岁。post-PV MF 32例(58.2%),post-ET MF 23例(41.8%)。诊断PV/ET至转化为MF的中位时间为7.8(1.1~23.4)年。所有患者中位随访37(1~156)个月,44例(80.0%)存活,11例(20.0%)死亡,中位生存时间为110.0(95% CI 87.5~132.8)个月。采用IPSS、DIPSS、DIPSS plus、IPSS-Chinese及MYSEC-PM对患者进行预后分组,各组患者的生存预后差异均无统计学意义(P> 0.05)。单因素分析示HGB<100 g/L是post-PV/ET MF患者的不良预后因素(P=0.003)。结论适用于PMF患者的IPSS、DIPSS、DIPSS plus、IPSS-Chinese及MYSEC-PM不能准确地对我国post-PV/ET MF患者进行预后分组。HGB<100 g/L是post-PV/ET MF患者的不良预后因素,可作为制定治疗方案的参考。

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