真性红细胞增多症和原发性血小板增多症后骨髓纤维化患者的预后研究

Analysis of prognostic factors in Chinese patients with post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis

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。

ObjectiveTo 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）.MethodsThe 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.ResultsFifty 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）.ConclusionIPSS, 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.