骨髓增生异常综合征患者血小板减少的预后意义初步研究

A preliminary study of prognostic value of thrombocytopenia in patients with primary myelodysplastic syndromes

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〈10×10^9／L）者256例（61．1％），严重减少（PLT〈30×10^9／L）者103例（24．6％）。PⅡ≥100×10^-9／L组、（50—99）×10^9／L组、（30～49）×10^9／L组和〈30×10^9／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〈30×10^9／L、红细胞平均体积（MCV）≤95fl、LDHI〉300U／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）一I及RAEB-11的患者较其他类型的患者预后差。多因素分析显示PLT〈30×10^9／L，MCV≤95fl，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〈30×10^91分，其他0分；MCV≤95fl 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RCMD1分，RAEB—I2分，RAEB一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。

Objective To investigate the prognostic value of thrombocytopenia in patients with pri- mary 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 evalu- ate factors that influence the prognosis. Results Two hundred and fifty-six cases （61.1% ） had thrombocyto- penia（PLT 〈 100×10^9/L）, one hundred and three cases （24.6%） had severe thrombocytopenia（PLT 〈 30 ×10^9/L）. Overall survival （OS） tended to shorten along with the decreasing of platelet count. Univariate analysis indicated that PLT 〈 30 ×10^9/L, MCV 〈95 fl, LDH 〉1300 U/L, lymphocyte-like mieromegakaryo- cyte, nucleated RBC PAS positive, IPSS cytogenetic intermediate- and poor-risk were all related with poor prognosis. Moreover, the prognosis of patients with RCMD, RAEB- I or RAEB- lI was poorer than that of the other subgroups. Among these parameters, PLT 〈 30 x 109/L, MCV 〈~95 fl, IPSS cytogenetic intermediate- and poor-risk group and RCMD, RAEB- I and RAEB- II had independent prognostic significance in multiva- riate analysis. Modified WPSS prognostic model was proposed by adopting PLT, MCV, chromosomal karyo- type 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 be- tween 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.