Objective: To evaluate the performances of the prognostic scoring systems devised for primary myelofibrosis(PMF)and the new developed MYSEC- PM(Mysec Prognostic Model)and investigate the risk factors in Chinese patients with post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis(post- PV/ET MF). The most widely used prognostic scoring systems in PMF included the International Prognostic Scoring System(IPSS), dynamic International Prognostic Scoring System(DIPSS), refined DIPSS(DIPSS plus), modified IPSS for Chinese(IPSS-Chinese), and modified DIPSS for Chinese(DIPSS- Chinese). Methods: The clinical and hematologic information of 55 consecutive patients diagnosed with post- PV/ET MF from March 1984 to December 2013 were retrospectively collected. All post-PV/ET MF patients were categorized according to IPSS, DIPSS, DIPSS plus, IPSS-Chinese, DIPSS-Chinese and MYSEC-PM, and the possible prognostic factors were statistically analyzed. Results: Fifty five patients diagnosed with post-PV MF(n=32)or post-ET MF(n=23)were analyzed with a median age of 59(range: 20- 88)years old, including 20 males and 35 females. Median time from original diagnosis to myelofibrosis was 7.8(range: 1.1- 23.4)years. With a median follow up from post-PV/ET MF diagnosis of 37(range: 1-156)months, 44(80.0%)patients were censored alive, 11(20.0%)patients died. Median survival was 110(95% CI 87.5-132.8)months. Using IPSS, DIPSS, DIPSS plus, IPSS- Chinese and MYSEC- PM criteria, there were no statistically significances in survival among different risk groups(P>0.05). In univariate analyses HGB<100 g/L(P=0.003)was the only factor associated with poorer overall survival. The prognosis in subjects with HGB≥100 g/L was significantly better than that with HGB<100 g/L(median OS: not reached vs 47 months, P=0.003). Conclusion: IPSS, DIPSS, DIPSS plus, IPSS- Chinese and MYSEC- PM did not accurately discriminate different risk categories in post PV/ET MF patients. HGB< 100 g/L was associated with poor outcome in post-PV/ET MF patients.
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。