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目的:比较分析骨髓增生异常综合征(MDS)FAB与WHO分型标准及疗效,探讨WHO分型标准具体应用的有关问题和临床实施意义。方法:采用WHO分型标准,对既往按FAB标准诊治的30例MDS再重新评定。结果:RCMD病情变化大,一般治疗效果差。RCMD、RAEB蛳1、RAEB蛳2、AL之间有演变规律,能反应或预示病情变化。随着MDS 亚型升级,体内正常造血克隆减少,骨髓造血功能衰竭是死亡原因之一。结论:两种方案分类结果差别较大。随着原始细胞比例的升高和增生异常的细胞系增多,骨髓正常造血功能减弱,治疗反应差,生存期缩短,转化为AL概率高。治疗方案的设计要权衡化疗获益和风险,采取个体化治疗。WHO标准更科学、细致,能较准确反应疾病临床特征,对临床治疗和预后有更大指导意义。某些亚型的临床意义仍需积累资料。
OBJECTIVE: To compare and analyze FAB and WHO classification criteria and efficacy of myelodysplastic syndrome (MDS) and discuss the relevant issues and clinical implications of specific application of WHO classification criteria. Methods: According to the WHO classification criteria, 30 MDS patients previously treated by FAB standard were re-evaluated. Results: The condition of RCMD changed greatly and the general treatment effect was poor. RCMD, RAEB 蛳 1, RAEB 蛳 2, AL have evolved between the law, can reflect or predict changes in condition. With the upgrade of MDS subtypes, normal hematopoietic clonality in vivo is reduced, and bone marrow hematopoietic failure is one of the causes of death. Conclusion: The results of the two schemes are quite different. With the increase of the proportion of primitive cells and the proliferation of abnormal cell lines, the normal bone marrow hematopoietic function weakened, the treatment response is poor, shorter survival, high probability of conversion to AL. The design of the treatment plan weighs the benefits and risks of chemotherapy and adopts individualized treatment. The WHO standard is more scientific and meticulous, which can reflect the clinical features of the disease more accurately and has greater guiding significance for clinical treatment and prognosis. The clinical significance of some subtypes still need to accumulate data.