title: Unique features of primary myelofibrosis in Chinese.
abstract: Clinical and laboratory features of 642 consecutive Chinese subjects with primary myelofibrosis (PMF) were analyzed and compared with those of 1054 predominately white subjects with PMF. Chinese subjects were significantly younger, fewer had constitutional symptoms, and fewer had a palpable spleen or liver. Anemia, in contrast, was significantly more common in Chinese as was an increased white blood cell count and low platelet count. The reason for these differences is unclear, but it does not seem to be correlated with delayed diagnosis. A small but significantly increased proportion of Chinese had the JAK2(V617F) mutation but no difference in the frequency of haplotypes associated with PMF in whites. Survival of Chinese with PMF was also significantly longer than that of whites with PMF. We found commonly used staging systems for PMF such as the International Prognostic Scoring System and the Dynamic International Prognostic Scoring System were suboptimal predictors of survival in Chinese with PMF, and we developed a revised prognostic score that should help in comparison of data between studies of PMF in different populations and planning of clinical trials.
authors.affiliation: MDS and MPN Centre, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.
authors.forename: Zefeng
authors.initials: Z
authors.lastname: Xu
authors.forename: Robert Peter
authors.initials: RP
authors.lastname: Gale
authors.forename: Yue
authors.initials: Y
authors.lastname: Zhang
authors.forename: Tiejun
authors.initials: T
authors.lastname: Qin
authors.forename: Huishu
authors.initials: H
authors.lastname: Chen
authors.forename: Peihong
authors.initials: P
authors.lastname: Zhang
authors.forename: Tianjiao
authors.initials: T
authors.lastname: Zhang
authors.forename: Liu
authors.initials: L
authors.lastname: Liu
authors.forename: Shiqiang
authors.initials: S
authors.lastname: Qu
authors.forename: Zhijian
authors.initials: Z
authors.lastname: Xiao
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000740:Anemia / Q000209:etiology; D044466:Asian People / Q000235:genetics*; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006239:Haplotypes / Q000235:genetics*; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D055728:Primary Myelofibrosis / Q000235:genetics* / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D015996:Survival Rate; D044465:White People / Q000235:genetics; D055815:Young Adult
chemical_list: C507924:JAK2 protein, human; D053614:Janus Kinase 2