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目的:分析世界卫生组织(WHO)与法-美-英协作组(FAB)2种标准分型结果的不同点,探讨WHO分型标准临床应用价值。方法:选择179例骨髓增生异常综合征(MDS)患者,其中168例是2003年~2006年确诊的原发性MDS,11例有血细胞减少伴有病态造血的病例。对179例患者按FAB与WHO2种分型方案重新进行评价。结果:按FAB分型标准:RA50例,RAS9例,RAEB62例,RAEB-T23例,CMML24例,11例未明确诊断,只描述了形态学特点。按WHO分型标准:RA14例,RAS5例,RCMD36例,RCMD-RS4例,MDS-U5例,6例不能确诊。结论:2种分型方案有较大差异,由于WHO分型中RA只限于贫血,单纯红系病态造血;将2系以上血细胞减少,2系以上病态造血,原始细胞<5%的病例归入了WHO新的亚型RCMD。通过本组病例分析:RCMD介于RA与RAEB中间,原始细胞不增多与RA相似,临床症状、实验室检查、血细胞形态学特点与RAEB相似。WHO将RAEB根据原始细胞数量分为两型,RAEB-T归入急性白血病,CMML归入骨髓增殖性疾病中,更符合临床的实际需要,有利于临床医师对治疗方案的选择。WHO分型方案仍需补充、修正、给血液学工作者提供更为完善的诊断标准。
OBJECTIVE: To analyze the differences between the two standard typing results of World Health Organization (WHO) and French-American-British Cooperative Group (FAB) and discuss the clinical value of the WHO classification criteria. Methods: A total of 179 patients with myelodysplastic syndrome (MDS) were selected. Among them 168 patients were diagnosed as primary MDS from 2003 to 2006, and 11 patients with cytopenia and cytopenia. 179 patients were re-evaluated according to the FAB and WHO2 genotyping schemes. Results: According to FAB classification criteria, there were only 50 cases of RA, 9 cases of RAS, 62 cases of RAEB, 23 cases of RAEB-T, 24 cases of CMML and 11 cases without clear diagnosis. According to WHO classification criteria: RA14 cases, RAS5 cases, RCMD36 cases, RCMD-RS4 cases, MDS-U5 cases, 6 cases can not be diagnosed. Conclusions: There are great differences between the two subtypes. Because RA is limited to anemia in erythropoiesis in WHO classification, erythroid mesenchymal hematopoiesis is purely defined. Cases of hematopoiesis of more than 2 lines, morbid hematopoiesis of more than 2 lines, and less than 5% of blasts The WHO new subtype of RCMD. Through the analysis of this group of patients: RCMD intermediate between RA and RAEB, no increase in primitive cells and RA similar to clinical symptoms, laboratory tests, blood cell morphological characteristics and RAEB similar. WHO divided RAEB into two types based on the number of primary cells, RAEB-T into acute leukemia, CMML into myeloproliferative diseases, more in line with the actual needs of the clinic, is conducive to the choice of treatment options for clinicians. The WHO classification scheme still needs to be supplemented and revised to provide hematology workers with more complete diagnostic criteria.