【摘要】 目的 　探索小儿骨髓增生异常综合征 (MDS)演变和转化规律。方法 　选自1 991年 2月～ 2 0 0 2年 1 2月 34例住院患儿按MDS诊断及疗效标准判断。结果 　 34例MDS中RA 1 9例 ,RAS 3例 ,RAEB 7例 ,RAEB -t5例。 1 9例RA中 2例转变为急性白血病 ;分别为M6和M5b ,演变时间为 7个月、6个月。 3例RAS中 1例演变为RAEB ,演变时间为 9个月。 7例RAEB中 1例演变为M2a ,演变时间为 2个月 ;2例演变为M4 ,演变时间分别为 1个月和 3个月 ;2例演变为RAEB -t,演变时间分别为 3个月和 5个月 ;1例缓解演变为RA。结论 　MDS患儿骨髓中原始细胞增多者易发生白血病 ,由MDS转化而来的急性白血病对化疗不敏感 ,生存期短 ,预后极差。MDS患儿外周血中有幼稚细胞者预后不良。更多还原

【Abstract】 Objective To investigate the law of evolvement and transformation of myeloproliferative disorders syndrome (MDS). Methods Cases selected from the patients hospitalized from February in 1991 to December in 2002,of which were diagnosed according to the diagnostic standard for MDS,blood picture and marrow picture proof,indicated no effect treated by folic acid,vitamin B_ 12 agent and other blood-produced medicine. Result It found that there were 19 AR,3 RAS,7 RAEB,5 RAEB-T among 34 MDS. 2 of 19 AR transformed into acute leukemia,among which,one was pattern M6 leukemia with 7 month- transforming periods,and pattern M5b leukemia in 6 months. 1 of 3 RAS changed into RAEB with 9 month-transforming periods. Among 7 RAEB,1 changed into pattern M4 leukemia in 2 months,2 into M4 in 1 and 3 months respectively,2 into RAEB-T within 3 and 5 months respectively,and 1 reverted to RA. Conclusion The more original former;primary cells found in marrow picture in cases with MDS ,the easier transformed into leukemia. This kind of leukemia was not sensitive to chemical therapy,had a poor prognosis and a short lifespan. It also shows that immature cell found in circulation of MDS clients is an indicator for poor prognosis.更多还原