CAJ | 학술논문

目的了解急性早幼粒细胞白血病(APL)临床及实验室特征.方法回顾分析我院近20年来诊治的513例APL患者的资料,包括患者年龄、性别、治疗前血象、疗效及随访等临床特征和APL形态学、免疫学、细胞遗传学及分子生物学(MICM)实验室资料.结果 APL中位发病年龄为33岁,男女比例为1.21:1.初诊中位白细胞数为4.3×109/L,外周血涂片分类异常早幼粒细胞检出率为85.8%;免疫表型与除APL外急性髓系白血病相比较,CDⅢ、CD34、HLA-DR、CD7、CD14及CD19的表达率低(P<0.01).CD2、CD33及MPO表达则高于其他急性髓系白血病组(P<0.01).细胞遗传学检查结果显示t(15;17)特异性染色体异常者占91.7%,其中t(15;17)标准易位占75.9%,t(15;17)伴附加染色体异常占15.8%,正常核型占7.5%,简单易位及复杂易位分别占0.4%.PML/RARα基因检出率为99.6%.APL患者完全缓解(CR)率为84.7%,DIC发生率13.4%.长期随访患者中,持续CR占75.9%,5年生存率为30.7%.CD34、CD2表达及细胞遗传学类型未缓解及CR二组无区别(P>0.05).未缓解组中位白细胞数高于CR组(P<0.01).结论 MICM综合分析对APL的诊断及判断预后均具有重要意义.外周血高白细胞数患者CR率低.
목적 료해급성조유립세포백혈병(APL)림상급실험실특정.방법 회고분석아원근20년래진치적513례APL환자적자료,포괄환자년령、성별、치료전혈상、료효급수방등림상특정화APL형태학、면역학、세포유전학급분자생물학(MICM)실험실자료.결과 APL중위발병년령위33세,남녀비례위1.21:1.초진중위백세포수위4.3×109/L,외주혈도편분류이상조유립세포검출솔위85.8%;면역표형여제APL외급성수계백혈병상비교,CDⅢ、CD34、HLA-DR、CD7、CD14급CD19적표체솔저(P<0.01).CD2、CD33급MPO표체칙고우기타급성수계백혈병조(P<0.01).세포유전학검사결과현시t(15;17)특이성염색체이상자점91.7%,기중t(15;17)표준역위점75.9%,t(15;17)반부가염색체이상점15.8%,정상핵형점7.5%,간단역위급복잡역위분별점0.4%.PML/RARα기인검출솔위99.6%.APL환자완전완해(CR)솔위84.7%,DIC발생솔13.4%.장기수방환자중,지속CR점75.9%,5년생존솔위30.7%.CD34、CD2표체급세포유전학류형미완해급CR이조무구별(P>0.05).미완해조중위백세포수고우CR조(P<0.01).결론 MICM종합분석대APL적진단급판단예후균구유중요의의.외주혈고백세포수환자CR솔저.
Objective To investigate the clinical and laboratory features of acute promyelocytic leukemia (APL).Methotis 513 APL patients in the last two decades were retrospectively analyzed in this research.We investigated the clinical features including age,sex,abnormality of peripheral hemogram before treatment.therapeutic effect and follow-up and laboratory data such as morphology,immunology,cytogenetics and molecular biology(MICM).Results The median age of the APL patients was 33 years old and the ratio of male and female was 1.21:1.Before treatment,the median level of WBC was 4.3×109/L and the deteetion rate of abnormal promyelocyte on blood film was 85.8%;with immunophenotypie detection,the expression levels of CD117、CD34、HLA-DR、CD7、CD14 and CD19 in APL were found to be lower and the expression 1evels of CD2、CD33 and MPO higher than those in other subtypes of acute myelocytie leukemia(AML)(beth P<0.01).Specific abnormal chromosome t(15;17)was detected in 91.7%of the patients,of whom 75.9%had standard translocation of t(15;17),being the most common one and 15.8% of the patients had t(15;17)with additional abnormal chromosome.There was only 7.5%of the patients with nolnlal karyotype.However,the presence of both simple translocation and complex translocation was seldom seen.With molecular biological detection.PML/RARα fusion gene positive rate was 99.6%.In a relativelv long clinical follow-up,we found that the complete remission(CR)rate in APL patients was 84.7%.incidence of DIC was 13.4%and five-year survival rate was 30.7%.111e median count of WBC in CR group was lower than that non-remission group(P<0.01).There were no significant differences on expressions of CD34 and CD2 and changes of cytogenetics between the two groups(P>0.05).Conclusions Comprehensive evaluation of MICM could be of important significance in the diagnosis and prognosis iudgrnent for APL patients.The CR rate in these patients with high WBC eount was considerable low.