Ghariani Ines, Braham Najia, Hassine Mohsen, Kortas Mondher
Laboratoire d'hématologie, CHU Farhat Hached, Sousse, Tunisia.
Ann Biol Clin (Paris). 2013 Mar-Apr;71(2):139-44. doi: 10.1684/abc.2013.0804.
Myelodysplastic syndromes (MDS) are myeloid disorders with various clinical and biological presentations. The French-American-British (FAB-1982) classification included five categories basing on morphology and bone marrow blast count. Three criteria are taken into account: 1) the percentage of blasts in peripheral blood and bone marrow, 2) the percentage of ringed sideroblasts, and 3) the number of monocytes in peripheral blood. The World Health Organization classification (WHO 2001, 2008) modifies the FAB system by also taking cytogenetic characteristics and molecular biology into consideration. The last classification (WHO-2008) takes into account: 1) the number of peripheral cytopenia, 2) the percentage of blasts in peripheral blood and bone marrow, 3) the percentage of ringed sideroblasts, 4) the possible presence of Auer Rods, and 5) the detection of a cytogenetic abnormality (the isolated 5q deletion). The following subgroups are defined: refractory cytopenia with unilineage dysplasia, refractory anemia with ringed sideroblasts, refractory cytopenia with multilineage dysplasia, refractory anemia with excess blasts, myelodysplastic syndrome unclassifiable and myelodysplastic syndrome with isolated del(5q).
骨髓增生异常综合征(MDS)是一类具有多种临床和生物学表现的髓系疾病。法美英(FAB-1982)分类基于形态学和骨髓原始细胞计数包括五个类别。考虑三个标准:1)外周血和骨髓中原始细胞的百分比,2)环形铁粒幼细胞的百分比,3)外周血中单核细胞的数量。世界卫生组织分类(WHO 2001、2008)通过同时考虑细胞遗传学特征和分子生物学对FAB系统进行了修改。最新分类(WHO-2008)考虑:1)外周血细胞减少的数量,2)外周血和骨髓中原始细胞的百分比,3)环形铁粒幼细胞的百分比,4)Auer小体的可能存在,5)细胞遗传学异常(孤立的5q缺失)的检测。定义了以下亚组:难治性单系发育异常性血细胞减少、环形铁粒幼细胞性难治性贫血、多系发育异常性难治性血细胞减少、原始细胞增多的难治性贫血、无法分类的骨髓增生异常综合征和孤立性del(5q)骨髓增生异常综合征。