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

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.