Validation of the WHO proposals for a new classification of primary myelodysplastic syndromes: a retrospective analysis of 1600 patients
Validation of the WHO proposals for a new classification of primary myelodysplastic syndromes: a retrospective analysis of 1600 patients
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DOI：
10.1016/s0145-2126(00)00088-6
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发表时间：
2000-12-01
影响因子：
2.7
通讯作者：
Aul, C
中科院分区：
文献类型：
--
作者：
Germing, U;Gattermann, N;Aul, C
1982年，法国-美国-英国（FAB）合作小组提出了骨髓增生异常综合征（MDS）的分类，其基于血液和骨髓中的形态学特征，即骨髓和外周原始细胞计数、奥尔杆、环形铁粒幼细胞和外周血中单核细胞的数量。该分类已用于许多关于MDS形态学、预后和治疗的研究。这种形态学分类的一些细节仍不清楚，一些患者无法分类。世界卫生组织（WHO）的一个工作组最近提出了一个新的MDS分类，该分类基于对FAB原始建议的重大修改。CMML和RAEB-T从MDS分类中删除，RAEB分为两组，髓母细胞计数低于和高于10%。此外，还定义了一组髓母细胞少于5%但有多系发育不良证据的患者。MDS患者5 q-作为唯一的染色体异常也被认为是一个单独的组。本研究的目的是验证新的分类预后的重要性，并将其与细胞遗传学和血液学特征在一个大系列的患者（n = 1600）的长期随访。我们能够证实RAEB I和RAEB II之间的预后有显著差异，以及难治性贫血和多系发育不良之间的差异。此外，5 q异常的患者比其他WHO亚型的患者预后好得多，但这仅适用于髓母细胞计数低于5%的患者。总之，WHO分类似乎定义了在预后方面比FAB亚型更同质的形态学亚组。(C)2000爱思唯尔科技有限公司版权所有。
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. (C) 2000 Elsevier Science Ltd. All rights reserved.