Germing U, Gattermann N, Strupp C, Aivado M, Aul C
Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Moorenstr. 5, 40225, Düsseldorf, Germany.
Leuk Res. 2000 Dec;24(12):983-92. doi: 10.1016/s0145-2126(00)00088-6.
In 1982, the French-American-British (FAB) cooperative group proposed a classification of myelodysplastic syndromes (MDS) based on morphological features in blood and bone marrow, namely on medullary and peripheral blast count, Auer rods, ring sideroblasts and the number of monocytes in the peripheral blood. This classification has been used for numerous studies regarding morphology, prognosis and treatment of MDS. Some details of this morphological classification remained unclear, and some patients were unclassifiable. A working group of the World Health Organization (WHO) recently proposed a new classification of MDS, based on a significant modification of the original FAB proposals. CMML and RAEB-T were removed from the MDS classification and RAEB was split into two groups with medullary blast counts below and above 10%. In addition, a group of patients with less than 5% medullary blasts but evidence of multilineage dysplasia was defined. MDS patients with 5q- as the sole chromosomal anomaly were also considered a separate group. The aim of the present study was to validate the new classification with respect to prognostic importance, and to correlate it with cytogenetic and hematological features in a large series of patients (n=1600) with a long-term follow up. We were able to confirm a significant difference in prognosis between RAEB I and RAEB II, as well as a difference between refractory anemia and multilineage dysplasia. Furthermore, patients with 5q- anomaly had a much better prognosis than other WHO subtypes, but this was only true for patients with a medullary blast count below 5%. In summary, the WHO classification appears to define morphological subgroups that are more homogeneous with respect to prognosis than the FAB subtypes.
1982年，法美英（FAB）协作组基于血液和骨髓的形态学特征，即髓系和外周血原始细胞计数、奥氏小体、环形铁粒幼细胞以及外周血单核细胞数量，提出了骨髓增生异常综合征（MDS）的分类方法。该分类已用于众多关于MDS形态学、预后及治疗的研究。这种形态学分类的一些细节仍不明确，且部分患者无法分类。世界卫生组织（WHO）的一个工作组最近基于对原FAB提议的重大修改，提出了MDS的新分类。慢性粒单核细胞白血病（CMML）和转化型原始细胞过多难治性贫血（RAEB-T）从MDS分类中移除，RAEB被分为两组，髓系原始细胞计数低于和高于10%。此外，定义了一组髓系原始细胞少于5%但有多系发育异常证据的患者。以5q-作为唯一染色体异常的MDS患者也被视为一个单独的组。本研究的目的是验证新分类在预后重要性方面的有效性，并将其与一大系列长期随访患者（n = 1600）的细胞遗传学和血液学特征相关联。我们能够证实难治性贫血伴原始细胞增多I型（RAEB I）和难治性贫血伴原始细胞增多II型（RAEB II）之间预后存在显著差异，以及难治性贫血和多系发育异常之间的差异。此外，5q-异常的患者预后比其他WHO亚型好得多，但这仅适用于髓系原始细胞计数低于5%的患者。总之，WHO分类似乎定义了在预后方面比FAB亚型更具同质性的形态学亚组。
