Müller-Berndorff H, Haas P S, Kunzmann R, Schulte-Mönting J, Lübbert M
Department of Hematology/Oncology, Albert-Ludwigs-University, Freiburg, Germany.
Ann Hematol. 2006 Aug;85(8):502-13. doi: 10.1007/s00277-005-0030-z. Epub 2006 May 20.
We retrospectively studied 89 consecutive patients diagnosed with primary myelodysplastic syndrome (MDS) over a period of 10 years to (1) identify prognostic factors for overall survival (OS) and leukemia-free survival (LFS); (2) to assess and compare the Bournemouth-, Spanish-, Düsseldorf-, Lille-, and the International prognostic scoring systems (IPSS); and to (3) compare the French-American-British (FAB) and World Health Organization (WHO) classifications. The median age of patients was 63 years (range, 26-85). Karyotype analyses were done in 85 patients (96%). Median OS was 3 years; 67 patients (75%) have died, and 28 (31%) had progression to acute myeloid leukemia (AML). Major independent prognostic variables for both OS and LFS (multivariate analysis) were percentage of bone marrow (BM) blasts (P < 0.0001), and in patients with cytogenetic data available, cytogenetic risk groups by Lille-score (OS, P = 0.031/LFS, P = 0.002) and IPSS (OS, P = 0.024). All five prognostic scoring systems successfully discriminated risk groups as regards OS and LFS, but in patients with cytogenetic data available, the major independent prognostic score for OS (P < 0.0001) and LFS (P = 0.006) was the IPSS. The FAB and WHO classifications also successfully discriminated between risk groups. The new WHO subgroups [refractory cytopenia with multilineage dysplasia (RCMD), with (RCMD-RS) or without ringed sideroblasts] showed a significantly (P = 0.0454) different prognosis for OS, but not for LFS (P = 0.0839), in comparison to the subgroups having erythroid dysplasia only (RA/RARS). Risk stratification into refractory anemia with excess blast-I (RAEB-I) and RAEB-II tended to yield different prognoses for OS and LFS. The 5q-minus syndrome strongly predicted for a good prognosis. In patients treated with the demethylating agent decitabine (n = 24), IPSS "poor risk" cytogenetics were unable to predict for the expected worse prognosis when compared to "intermediate-risk" cytogenetics. In conclusion, we confirm in a single-center patient cohort that the use of the WHO classification improves the predictive value of the FAB classification and that, in patients with cytogenetic data available, the IPSS can be used for clinical decision-making.
我们回顾性研究了连续89例在10年期间被诊断为原发性骨髓增生异常综合征（MDS）的患者，目的是：（1）确定总生存期（OS）和无白血病生存期（LFS）的预后因素；（2）评估和比较伯恩茅斯、西班牙、杜塞尔多夫、里尔及国际预后评分系统（IPSS）；以及（3）比较法美英（FAB）和世界卫生组织（WHO）的分类。患者的中位年龄为63岁（范围26 - 85岁）。85例患者（96%）进行了核型分析。中位OS为3年；67例患者（75%）死亡，28例（31%）进展为急性髓系白血病（AML）。OS和LFS的主要独立预后变量（多变量分析）是骨髓（BM）原始细胞百分比（P < 0.0001），对于有细胞遗传学数据的患者，根据里尔评分（OS，P = 0.031/LFS，P = 0.002）和IPSS（OS，P = 0.024）划分的细胞遗传学风险组。所有五个预后评分系统在OS和LFS方面都成功区分了风险组，但对于有细胞遗传学数据的患者，OS（P < 0.0001）和LFS（P = 0.006）的主要独立预后评分是IPSS。FAB和WHO分类也成功区分了风险组。与仅具有红系发育异常的亚组（RA/RARS）相比，新的WHO亚组[伴有（RCMD-RS）或不伴有环形铁粒幼细胞的多系发育异常难治性血细胞减少症（RCMD）]的OS预后有显著差异（P = 0.0454），但LFS无差异（P = 0.0839）。分为难治性贫血伴过多原始细胞-I（RAEB-I）和RAEB-II的风险分层在OS和LFS方面往往产生不同的预后。5q-综合征强烈预示预后良好。在接受去甲基化药物地西他滨治疗的患者（n = 24）中，与“中危”细胞遗传学相比，IPSS“高危”细胞遗传学无法预测预期的更差预后。总之，我们在一个单中心患者队列中证实，使用WHO分类提高了FAB分类的预测价值，并且对于有细胞遗传学数据的患者，IPSS可用于临床决策。
