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骨髓增生异常综合征:一种具有预后意义的评分系统。

Myelodysplastic syndromes: a scoring system with prognostic significance.

作者信息

Mufti G J, Stevens J R, Oscier D G, Hamblin T J, Machin D

出版信息

Br J Haematol. 1985 Mar;59(3):425-33. doi: 10.1111/j.1365-2141.1985.tb07329.x.

DOI:10.1111/j.1365-2141.1985.tb07329.x
PMID:3970861
Abstract

141 patients with MDS were classified according to the FAB criteria and followed up for a period of 4-192 months. It was recognized that patients with RAEBT had a uniformly poor prognosis. However, there was a wide variation in survival among the other subgroups. A score of 1 was assigned to each of the following presenting haematological features: bone marrow blasts greater than or equal to 5%, platelets less than or equal to 100 X 10(9)/l, neutrophils less than or equal to 2.5 X 10(9)/l and Hb less than or equal to 10.0 g/dl. Therefore the score for each patient ranged between 0 and 4. There were no statistically significant differences between those patients who scored 0 or 1, or between those who scored 2 and 3. Therefore patients were put into three groups: Group A (score 0 or 1), Group B (score 2 or 3), Group C (score 4). The differences in survival between each of the three groups are highly significant (P less than 0.00001). This system further separates patients with RA, RAS, RAEB into good and bad prognostic groups. This study also confirms that deaths due to cytopenias are more common than those due to transformation to AML. The use of this scoring system in conjunction with the FAB criteria for MDS should serve as a prognostic tool on which to base treatment.

摘要

141例骨髓增生异常综合征(MDS)患者根据FAB标准进行分类,并随访4至192个月。已认识到转化型原始细胞过多难治性贫血(RAEBT)患者的预后普遍较差。然而,其他亚组的生存期差异很大。对以下每个血液学特征表现评分为1分:骨髓原始细胞大于或等于5%、血小板小于或等于100×10⁹/L、中性粒细胞小于或等于2.5×10⁹/L以及血红蛋白小于或等于10.0g/dl。因此,每位患者的评分在0至4分之间。评分为0或1分的患者之间,以及评分为2和3分的患者之间,均无统计学显著差异。因此,患者被分为三组:A组(评分0或1分)、B组(评分2或3分)、C组(评分4分)。三组之间的生存期差异非常显著(P<0.00001)。该系统进一步将难治性贫血(RA)、环形铁粒幼细胞性难治性贫血(RAS)、转化型原始细胞过多难治性贫血(RAEB)患者分为预后良好和不良组。本研究还证实,因血细胞减少导致的死亡比转化为急性髓系白血病(AML)导致的死亡更常见。将该评分系统与MDS的FAB标准结合使用,应作为制定治疗方案的预后工具。

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