急性红白血病的生物学特点及初步疗效的临床分析

Biological characteristics and primary therapeutic response of acute erythroid leukemia

目的 探讨急性红白血病（AEL）的生物学特点,并对初步治疗的疗效进行分析.方法 回顾分析28例初治AEL患者的临床资料,其中伴多系病态造血及无多系病态造血患者各14例,针对其形态学、免疫学、细胞遗传学和分子生物学特征及初次诱导治疗完全缓解（CR）率进行分析.结果 28例初治AEL患者中有14例（50％）伴多系病态造血,累及二系或三系；6例行免疫分型检测,高表达髓系原始细胞相关抗原；8例行染色体核型分析,5例核型异常,其中4例为复杂核型,1例伴有＋8染色体；4例行WT1分子检测均为阳性；诱导治疗CR率为39.29％（11/28）,伴多系病态造血组为35.71％（5/14）,无多系病态造血组为42.86％（6/14）,两组间差异无统计学意义（P＞0.05）.复杂核型组为25.00％（1/4）,预后中等核型组为50.00％（2/4）.结论 AEL具有独特的生物学特征：伴有较高的多系病态造血发生率；异常核型检出率高,且多为累及5号和（或）7号染色体的复杂核型,复杂核型组CR率低；对化疗CR低,治疗效果差.

Objective To observe the biological characteristics and analyse primary therapeutic response of acute erythroid leukemia.Methods The data of 28 patients primarily diagnosed as acute erythroid leukemia were analyzed.The patients were divided into with muhilineage dysplasia group and without muhilineage dysplasia group,and the morphology,immunology,cytogenetics and molecular biology characteristics and the complete remission rate of the first induction therapy were compared.Results There were 14 cases（50％）with muhilineage dysplasia,which involved in two lineage or trilineage.In 6 cases by flow cytometry,the myeloid blast immunophenotypes were common expressed.In 8 cases detected by karyotype analysis,5 cases were chromosomal abnormal,including 4 cases were complex chromosomal abnormal,1 case was trisomy 8.In 4 cases underwent WT1 detection,all of them were positive.The complete remission rate of the first induction therapy was 39.29％（11/28）,the ratein the multilineage dysplasia group was 35.71％（5/14）,and the ratein without multilineage dysplasia group was 42.86％（6/14）,the difference had no statistical significance（P＞0.05）.The complete remission rate of the complex chromosome group was 25.00％（1/4）,the intermediate prognostic group was 50.00％（2/4）.Conclusions Acute erythroid leukemia had special biological features different from other subtype AML：accompanyed with high frequency of multilineage dysplasia.The abnormality of karyotype were high,and it was often complex karyotype involved with chromosome 5 and/or chromosome 7,which had a low complete remission rate.The complete remission rate of chemotherapy was low,treatment effect was poor.