Chen M, Xu Z F, Xu J Q, Li B, Zhang P H, Qin T J, Zhang Y, Wang J Y, Zhang H L, Fang L W, Pan L J, Hu N B, Qu S Q, Xiao Z J
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, the State Key Laboratory of Experimental Hematology, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 Oct 14;37(10):876-880. doi: 10.3760/cma.j.issn.0253-2727.2016.10.012.
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). 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. Fifty five patients diagnosed with post-PV MF(=32)or post-ET MF(=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% 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(>0.05). In univariate analyses HGB<100 g/L(=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, =0.003). 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.
评估为原发性骨髓纤维化（PMF）设计的预后评分系统以及新开发的MYSEC-PM（Mysec预后模型）的性能，并调查真性红细胞增多症后骨髓纤维化和原发性血小板增多症后骨髓纤维化（真性红细胞增多症/原发性血小板增多症后骨髓纤维化）中国患者的危险因素。PMF中使用最广泛的预后评分系统包括国际预后评分系统（IPSS）、动态国际预后评分系统（DIPSS）、改良DIPSS（DIPSS plus）、中国版改良IPSS（IPSS-Chinese）和中国版改良DIPSS（DIPSS-Chinese）。回顾性收集了1984年3月至2013年12月连续诊断为真性红细胞增多症/原发性血小板增多症后骨髓纤维化的55例患者的临床和血液学信息。所有真性红细胞增多症/原发性血小板增多症后骨髓纤维化患者根据IPSS、DIPSS、DIPSS plus、IPSS-Chinese、DIPSS-Chinese和MYSEC-PM进行分类，并对可能的预后因素进行统计学分析。分析了55例诊断为真性红细胞增多症后骨髓纤维化（n=32）或原发性血小板增多症后骨髓纤维化（n=23）的患者，中位年龄为59岁（范围：20-88岁），包括20例男性和35例女性。从最初诊断到骨髓纤维化的中位时间为7.8年（范围：1.1-23.4年）。从真性红细胞增多症/原发性血小板增多症后骨髓纤维化诊断开始的中位随访时间为37个月（范围：1-156个月），44例（80.0%）患者存活，11例（20.0%）患者死亡。中位生存期为110个月（95%CI 87.5-132.8）。使用IPSS、DIPSS、DIPSS plus、IPSS-Chinese和MYSEC-PM标准，不同风险组之间的生存率无统计学意义（P>0.05）。单因素分析中，血红蛋白<100 g/L（P=0.003）是唯一与总体生存率较差相关的因素。血红蛋白≥100 g/L的患者预后明显优于血红蛋白<100 g/L的患者（中位总生存期：未达到vs 47个月，P=0.003）。IPSS、DIPSS、DIPSS plus、IPSS-Chinese和MYSEC-PM不能准确区分真性红细胞增多症/原发性血小板增多症后骨髓纤维化患者的不同风险类别。血红蛋白<100 g/L与真性红细胞增多症/原发性血小板增多症后骨髓纤维化患者的不良预后相关。
