骨髓增生异常综合征临床分析  

Clinical Analysis of Myelodysplastic Syndrome

作　　者：熊梅[1] 朱焕玲[1] 谢莉萍[1] 汪宇春[1] 何川[1] 季杰[1] 廖小梅[1] 陈心传[1] 李建军[1] 崔旭[1] 龚玉萍[1] 刘霆[1] 

机构地区：[1]四川大学华西医院血液科,成都610041

出　　处：《华西医学》2013年第7期994-997,共4页West China Medical Journal

基　　金：中华医学会分子生物学临床应用研究专项基金资助(CAMB042010)~~

摘　　要：目的探讨骨髓增生异常综合征(MDS)患者的临床特点。方法选取我院2008年3月-2012年10月确诊为MDS的231例患者临床资料进行回顾性分析。患者年龄21～87岁,中位年龄59岁。结果 231例患者中,难治性血细胞减少伴多系发育异常(RCMD)最多见,占45.0%(104/231);以贫血乏力症状就诊多见占66.7%(154/231);血常规中以全血细胞均减少多见占61%(141例/231例);网织红细胞以正常或增高为主占61%(141/231);低荧光值增高多见62%(144/231)。乳酸脱氢酶和铁蛋白在各诊断亚型及各国际预后积分系统(IPSS)评分间存在差异,其中乳酸脱氢酶在难治性贫血伴原始细胞增多2型(RAEB-2)中高于综合组:难治性贫血(RA)、难治性贫血伴环状铁粒幼细胞(RAS)、5q综合征及RCMD相比较差异有统计学意义(P<0.05),高危组乳酸脱氢酶高于中危1组及中危2组,其差异有统计学意义(P<0.05),高危组铁蛋白高于中危1组其差异有统计学意义(P<0.05),其余差异无统计学意义(P>0.05)。染色体异常率为39%,其中20例为复杂染色体核型,IPSS评分中危1最多见为52.4%(55/105)。结论 MDS临床表现多样,缺乏特异性,需综合骨髓涂片、活检、细胞遗传学的结果提高诊断率。Objective To investigate the clinical features of myelodysplastic syndrome（MDS）.Methods A total of 231 cases of MDS diagnosed between March 2008 and October 2012 were analyzed retrospectively.Their age was between 21 and 87 years old,and the median age was 59 years.Results Among them,refractory cytopenia of multilineage dysplasia（RCMD） was the most common subtype,accounting for 45.0%（104/231）.Anemia and fatigue symptoms were common presentations,accounting for 66.7%（154/231）.A total of 141 patients（61%） showed pancytopenia with normal or slightly higher reticulocyte.Lactic dehydrogenase（LDH） and ferretin（sF） were different in various subtypes and IPSS scores.LDH in RAEB-2 was higher than comprehensive group（refractory anemia,refractory anemia with ringed sideroblasts,5 q-syndrome） and RCMD（P0.05）,and LDH in high-risk was higer than Intermediate-1 risk and Intermediate-2 risk（P0.05）.Ferretin in high-risk was higer than Intermediate-1 risk（P0.05）.In 105 patients with cytogenetic examination,41 patients（39%） had chromosome abnormalities.And the most frequent cytogenetic abnormality was complex chromosome.The proportion of Intermediate-1 risk was the highest in the IPSS,accounting for 52.4%（55/105）.Conclusions The clinical manifestation of MDS is presented as diversiform and lacks specificity.Combined bone marrow smear,biopsy,and cytogenetic can improve the diagnosis of MDS.

关 键 词：骨髓增生异常综合征 乳酸脱氢酶 铁蛋白 细胞遗传学 临床分析 

分 类 号：R551.3[医药卫生—血液循环系统疾病]