骨髓增生异常综合征WHO分型标准的临床应用

The clinical use of WHO classification system in MDS

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H5％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 explore the merit of clinical application of WHO classification system by analyzing the difference between WHO and FAB classification system. Method：179 cases between 2003-2006 were chosen and evaluated according to FAB and WHO classification system, which included 168 primary MDS, 11 cases with blood cells decreasing and dysplasia. Result： With FAB classification system, the different types of RA, RAS, RAEB, RAEB-T and CMML were 50, 9, 62, 23, respectively and 11 were uncertain only described in morphological. With WHO classification system： ones of RA, RAS, RCMD, RCMD-RAS and MDS-U were 14, 5, 36, 4, 5, respectively and 6 were uncertain. Conclusion：There were obvious differences between two classification system. In WHO classification system, the type of RA only defined anemia and unilineage erythoid dysplasia while the cases suffered from multilineage cytopenia, multilineage dysplasia and less than 5% blasts in bone marrow were clarified to a new subtype RCMD. According to the data obtained, RCMD was a mid-type between RA and RAEB, which were similar to RA in unincreased blasts and similar to RAEB in clinical syndrome, lab test and morphology. RAEB was divided into two subtypes according to the blasts in WHO classification system. RAEB-T was defined to AML and CMML was defined to hyperproliferative disease in WHO classification system, which was more fit to clinical requirement and helpful to choose therapy project. WHO classification system needed to be supplemented and corrected to provided better diagnostic criteria.