Lee J-H, Lee J-H, Shin Y-R, Lee J-S, Kim W-K, Chi H-S, Park C-J, Seo E-J, Lee K-H
Department of Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Leukemia. 2003 Feb;17(2):305-13. doi: 10.1038/sj.leu.2402798.
We retrospectively studied 227 patients with MDS (1) to identify the prognostic factors of survival and acute leukemia evolution in Korean patients with MDS, (2) to apply different prognostic scoring systems to the same group of patients, and (3) to compare the FAB with the WHO classification. Six scoring systems were applied to the patients, and the FAB and WHO classifications were compared. The patients' median age was 57 years. The median survival time was 21 months, and age, dysgranulopoiesis and the IPSS cytogenetic groups were independent prognostic factors for survival. Acute leukemia occurred in 34 patients, and the cumulative incidence was 27.1% at 3 years. Marrow blast percentage was the only independent prognostic factor for acute leukemia evolution. Most scoring systems successfully discriminated risk groups for survival and acute leukemia evolution, but patient distribution into risk groups varied according to the scoring systems. Refractory cytopenia with multilineage dysplasia and RAEB II seemed to have different prognoses from RA or RARS and RAEB I, respectively. In summary, our MDS patients had different disease natures from those of Western countries regarding clinical features, prognostic factors and cytogenetic profiles. Although the WHO classification seems to improve the FAB classification, further studies are warranted to validate the utility of the WHO classification before it is accepted for routine clinical use. Our study has the limitations of retrospective analysis, and our results should be verified in future prospective studies.
我们对227例骨髓增生异常综合征(MDS)患者进行了回顾性研究,(1)以确定韩国MDS患者生存及急性白血病演变的预后因素,(2)对同一组患者应用不同的预后评分系统,以及(3)比较FAB分类与WHO分类。对患者应用了六种评分系统,并比较了FAB和WHO分类。患者的中位年龄为57岁。中位生存时间为21个月,年龄、粒细胞生成异常及IPSS细胞遗传学分组是生存的独立预后因素。34例患者发生急性白血病,3年时累积发病率为27.1%。骨髓原始细胞百分比是急性白血病演变的唯一独立预后因素。大多数评分系统成功区分了生存及急性白血病演变的风险组,但患者在风险组中的分布因评分系统而异。伴有多系发育异常的难治性血细胞减少症和RAEB II的预后似乎分别与RA或RARS及RAEB I不同。总之,我们的MDS患者在临床特征、预后因素及细胞遗传学特征方面与西方国家患者具有不同的疾病性质。虽然WHO分类似乎改进了FAB分类,但在被接受用于常规临床使用之前,仍需进一步研究以验证WHO分类的实用性。我们的研究存在回顾性分析的局限性,我们的结果应在未来的前瞻性研究中得到验证。