CAJ | 학술논문

目的研究中国上海及周边地区原发性骨髓纤维化(PMF)患者临床特点和预后.方法回顾性分析1996年1月至2013年12月间我院收治的75例PMF患者的临床特征、实验室数据和生存情况,与国外白种人患者和我国其他中心黄种人患者数据进行比较,分析患者预后情况及可能影响黄种人生存的各种预后因素.分类变量的比较采用x2检验,生存分析采用Kaplan-Meier方法,预后的单因素分析采用Log-rank法,多因素分析采用COX回归模型.结果 75例患者中位年龄56(19～ 81)岁,51例(68％)患者HGB＜ 100 g/L,HGB中位数83 g/L,与我国其他中心黄种人患者相似,较白种人发病年龄轻、贫血比例高、贫血程度较严重,血小板偏低的患者亦较多.运用IPSS、动态的国际预后积分系统(DIPSS)分组比较患者生存,除低危组和中危-1组之间差异不明显外,其他各组差异均有统计学意义.在单因素分析中,全身症状、HGB＜ 100 g/L、HGB＜ 80 g/L、PLT≤1 00×109/L、WBC＜ 10× 109/L及外周血原始细胞≥0.01,均与不良预后呈显著相关.多因素分析中,IPSS和HGB＜ 80 g/L对生存仍有影响.结论黄种人PMF患者可能存在发病年龄轻、贫血更严重的特征.但IPSS、DIPSS仍可适用于我国患者,作为预后判断、分层治疗的重要依据.加大贫血程度在预后评分系统中的权重可能更好地对黄种人患者进行预后分层.
목적 연구중국상해급주변지구원발성골수섬유화(PMF)환자림상특점화예후.방법 회고성분석1996년1월지2013년12월간아원수치적75례PMF환자적림상특정、실험실수거화생존정황,여국외백충인환자화아국기타중심황충인환자수거진행비교,분석환자예후정황급가능영향황충인생존적각충예후인소.분류변량적비교채용x2검험,생존분석채용Kaplan-Meier방법,예후적단인소분석채용Log-rank법,다인소분석채용COX회귀모형.결과 75례환자중위년령56(19～ 81)세,51례(68％)환자HGB＜ 100 g/L,HGB중위수83 g/L,여아국기타중심황충인환자상사,교백충인발병년령경、빈혈비례고、빈혈정도교엄중,혈소판편저적환자역교다.운용IPSS、동태적국제예후적분계통(DIPSS)분조비교환자생존,제저위조화중위-1조지간차이불명현외,기타각조차이균유통계학의의.재단인소분석중,전신증상、HGB＜ 100 g/L、HGB＜ 80 g/L、PLT≤1 00×109/L、WBC＜ 10× 109/L급외주혈원시세포≥0.01,균여불량예후정현저상관.다인소분석중,IPSS화HGB＜ 80 g/L대생존잉유영향.결론 황충인PMF환자가능존재발병년령경、빈혈경엄중적특정.단IPSS、DIPSS잉가괄용우아국환자,작위예후판단、분층치료적중요의거.가대빈혈정도재예후평분계통중적권중가능경호지대황충인환자진행예후분층.
Objective To investigate the clinical and hematological features and prognostic factors of primary myelofibrosis (PMF) patients in Shanghai and surronding area of Shanghai,China.Methods The clinical manifestations,laboratory parameters and survival were retrospectively analyzed in 75 PMF patients diagnosed from Jan,1996 to Dec,2013 in our hospital and were compared with those of Chinese subjects from Tianjin and foreign Caucasian patients,respectively.Comparison of categorical variables was performed by x2 test.Survival was estimated using the Kaplan-Meier method.Log-rank test was used to compare survival date.A Cox model was used for multivariate analyses.Results The median age of the 75 patients was 56 (19-81) years old.There were 51 (68％) patients with HGB less than 100 g/L.The median value of HGB was 83 g/L.Similar with those from Tianjin,the patients in our study were significantly younger with higher proportion of severe anemia and lower platelet counts when compared with foreign Caucasian patients.Using IPSS and dynamic international prognostic scoring system (DIPSS)model,the survival curves of intermediate-1,intermediate-2 and high risk groups were significantly different.In univariate analyses,variables significantly correlated with poor prognosis were systemic symptoms,HGB ＜ 100 g/L,HGB ＜ 80 g/L,PLT≤100 × 109/L,WBC ＜ 10 × 109/L and blood blasts≥0.01.Multivariate analysis identified IPSS and HGB＜80 g/L as independent risk factors for survival.Conclusion Chinese PMF patients may have characteristics of younger age at onset with more severe anemia.However,IPSS and DIPSS model are still suitable for Chinese patients to predict survival.To increase the weight of anemia severity may provide a better prognostic stratification for Chinese patients with PMF.