CAJ | 학술논문

目的探讨血小板减少对判断骨髓增生异常综合征(MDS)患者预后的意义.方法回顾性分析419例原发性MDS患者临床资料,应用Kaplan-Meier、Log-rank检验及COX回归模型评估影响预后的因素.结果 419例MDS患者中血小板减少(PLT<100×109/L)者256例(61.1%),严重减少(PLT<30×109/L)者103例(24.6%).PLT≥100×109/L组、(50～99)×109/L组、(30～49)×109/L组和<30×109/L组患者中位生存时间分别为41、38、19和17个月,差异有统计学意义(P=0.000).单因素分析显示PLT<30×109/L、红细胞平均体积(MCV)≤95 fl、LDH≥300 U/L、有淋巴样小巨核细胞、有核红细胞糖原染色(E-PAS)阳性、国际预后积分系统(IPSS)染色体核型预后中等及不良为MDS不良预后因素;WHO分型中难治性血细胞减少伴有多系发育异常(RCMD)、难治性贫血伴有原始细胞过多(RAEB)-Ⅰ及RAEB-Ⅱ的患者较其他类型的患者预后差.多因素分析显示PLT<30×109/L,MCV≤95 fl,IPSS染色体核型预后中等及不良,WHO分型RCMD、RAEB-Ⅰ及RAEB-Ⅱ具有独立预后意义.修订的WHO分型预后积分系统(WPSS)各参数赋值如下:PLT <30×109/L 1分,其他0分; MCV≤95 fl 1分,其他0分;染色体核型良好0分,中等1分,不良2分;WHO分型中RCMD 1分,RAEB-Ⅰ2分,RAEB-Ⅱ3分,其他0分.患者分为低危(0～1分)、中危-1(2～3分)、中危-2(4～5分)和高危组(6～7分),其中位生存期分别为59、28、14和4个月,各组间差异具有统计学意义(P值均<0.05).结论血小板严重减少的MDS患者预后不良,结合血小板计数、染色体核型、MCV及修订的WPSS有望更好地促进我国MDS患者分组治疗策略的制订.
목적 탐토혈소판감소대판단골수증생이상종합정(MDS)환자예후적의의.방법 회고성분석419례원발성MDS환자림상자료,응용Kaplan-Meier、Log-rank검험급COX회귀모형평고영향예후적인소.결과 419례MDS환자중혈소판감소(PLT<100×109/L)자256례(61.1%),엄중감소(PLT<30×109/L)자103례(24.6%).PLT≥100×109/L조、(50～99)×109/L조、(30～49)×109/L조화<30×109/L조환자중위생존시간분별위41、38、19화17개월,차이유통계학의의(P=0.000).단인소분석현시PLT<30×109/L、홍세포평균체적(MCV)≤95 fl、LDH≥300 U/L、유림파양소거핵세포、유핵홍세포당원염색(E-PAS)양성、국제예후적분계통(IPSS)염색체핵형예후중등급불량위MDS불량예후인소;WHO분형중난치성혈세포감소반유다계발육이상(RCMD)、난치성빈혈반유원시세포과다(RAEB)-Ⅰ급RAEB-Ⅱ적환자교기타류형적환자예후차.다인소분석현시PLT<30×109/L,MCV≤95 fl,IPSS염색체핵형예후중등급불량,WHO분형RCMD、RAEB-Ⅰ급RAEB-Ⅱ구유독립예후의의.수정적WHO분형예후적분계통(WPSS)각삼수부치여하:PLT <30×109/L 1분,기타0분; MCV≤95 fl 1분,기타0분;염색체핵형량호0분,중등1분,불량2분;WHO분형중RCMD 1분,RAEB-Ⅰ2분,RAEB-Ⅱ3분,기타0분.환자분위저위(0～1분)、중위-1(2～3분)、중위-2(4～5분)화고위조(6～7분),기중위생존기분별위59、28、14화4개월,각조간차이구유통계학의의(P치균<0.05).결론 혈소판엄중감소적MDS환자예후불량,결합혈소판계수、염색체핵형、MCV급수정적WPSS유망경호지촉진아국MDS환자분조치료책략적제정.
Objective To investigate the prognostic value of thrombocytopenia in patients with primary myelodysplastic syndromes(MDS). Methods Four hundred and nineteen primary MDS patients were retrospectively analyzed. Kaplan-Meier method, Log-rank test and COX regression model were used to evaluate factors that influence the prognosis. Results Two hundred and fifty-six cases(61.1%) had thrombocytopenia(PLT<100×109/L), one hundred and three cases(24.6%) had severe hrombocytopenia(PLT<30×109/L). Overall survival (OS) tended to shorten along with the decreasing of platelet count. Univariate analysis indicated that PLT<30×109/L, MCV≤95 fl, LDH≥300 U/L, lymphocyte-like micromegakaryocyte, nucleated RBC PAS positive, IPSS cytogenetic intermediate- and poor-risk were all related with poor prognosis. Moreover, the prognosis of patients with RCMD, RAEB-Ⅰor RAEB-Ⅱwas poorer than that of the other subgroups. Among these parameters, PLT<30×109/L, MCV≤95 fl, IPSS cytogenetic intermediate- and poor-risk group and RCMD, RAEB-Ⅰ and RAEB-Ⅱ had independent prognostic significance in multivariate analysis. Modified WPSS prognostic model was proposed by adopting PLT, MCV, chromosomal karyotype and WHO classification. The OS of patients with low risk, intermediate-1 risk, intermediate-2 risk and high risk were 59, 28, 14 and 4 months, respectively, and there was a statistically significant difference between the groups (P<0.05). Conclusion Severe thrombocytopenia indicated unfavorable prognosis, in combination with MCV, chromosomal karyotype and WHO classification, a modified WPSS prognostic model was proposed and worked well for prognostic indication in patients with MDS.