【摘要】 目的:探讨骨髓增生异常综合征(MDS)患者转化为急性白血病(AL)的生物学特点及治疗反应。方法:采用WHO标准回顾性分析MDS转化为AML的临床特点,探讨影响MDS转化为急性髓系白血病(AML)的相关因素,分析转化为AML患者近期的治疗反应。结果:153例中高危MDS患者转化为AL 26例(17%),中位转化时间4(1-29)个月,均为急性髓系白血病(AM L),以AM L-M2、AM L-M4、AM L-M5、AM L-M6为主。转成的AM L患者均有不同程度的贫血症状,2例出现髓外浸润;26例转成的AM L中18例(69.2%)有染色体核型异常,同转化为AML前的核型相比,9例异常核型发生了改变(出现了新的核型或原有核型消失)。个体化治疗转成AM L的患者总有效率达80%,能使患者生存质量得以改善。结论:中高危M DS易进展为AM L,多种因素参与M DS转化为AM L;转成的AM L具有独特的生物学特性,且预后较差,去甲基化治疗可获得较好的近期疗效。更多还原

【Abstract】 Objective: To study the biological characteristics and treatment response of patients with myelodysplastic syndrome( MDS) transformed into acute leukemia( AL). Methods: Using WHO standard method,the clinical characteristics of patients with MDS into AML were retrospectively analyzed,the related factors influencing the MDS into AML and the treatment response of patients were analyzed. Results: Twenty-six cases( 17%) of MDS were transformed into AL among 153 cases of middle and high risk MDS,all of which were AML. The median time of transformation from MDS into AML was 4 months( 1-29),and these cases transformed into AML-M2,M4,M5 and M6. In these 26 cases of AML patients,the varying degrees of anemia symptom appeared,2 cases were with marrowinfiltration,18 cases( 69. 2%) were with abnormal chromosome karyotype. Compared with karyotype before transformation into AML,the abnormal karyotype in 9 cases had been conversed( newkaryotype or disappear once of existing karyotype). Total efficiency of individualized treatment for MDS transformed into AML was 80%. This treatment could improve the patients quality of life. Conclusion: Middle and high risk MDS patients are prone to be tranformed into AML. Multiple factors are involved in the transformation of MDS into AML. These patients showed the special biological characteristics and poorer prognosis. Demethylation treatment is helpful to achieve a good near-term curative effect.更多还原