title: [Analysis of prognostic factors in Chinese patients with post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis].
abstract: 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%CI87.5-132.8)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.
authors: Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, the State Key Laboratory of Experimental Hematology, Tianjin 300020, China.
authors: M
authors: M
authors: Chen
authors: Z F
authors: ZF
authors: Xu
authors: J Q
authors: JQ
authors: Xu
authors: B
authors: B
authors: Li
authors: P H
authors: PH
authors: Zhang
authors: T J
authors: TJ
authors: Qin
authors: Y
authors: Y
authors: Zhang
authors: J Y
authors: JY
authors: Wang
authors: H L
authors: HL
authors: Zhang
authors: L W
authors: LW
authors: Fang
authors: L J
authors: LJ
authors: Pan
authors: N B
authors: NB
authors: Hu
authors: S Q
authors: SQ
authors: Qu
authors: Z J
authors: ZJ
authors: Xiao
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D044466:Asian People; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011087:Polycythemia Vera; D055728:Primary Myelofibrosis; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013920:Thrombocythemia, Essential; D055815:Young Adult