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骨髓增生异常综合征的预后因素:五种评分系统分析

Prognostic factors in myelodysplastic syndromes: analysis of five scoring systems.

作者信息

Rubio-Felix D, Giraldo P, Franco E, Gimeno J, Giralt M

机构信息

Servicio de Hematologia-Hemoterapia, Hospital Miguel Servet, Zaragoza, Spain.

出版信息

Hematol Oncol. 1995 May-Jun;13(3):139-52. doi: 10.1002/hon.2900130304.

Abstract

PURPOSE

To evaluate the prognostic significance of five scoring systems applied with predictive trends to myelodysplastic syndromes (MDS).

PATIENTS AND METHODS

This study was comprised of 226 patients with MDS diagnosed in accordance with the FAB criteria and followed up in our department between January 1975 and April 1992. The following MDS subtypes were found: refractory anaemia (RA), 59 cases; refractory sideroblastic anaemia (RSA), 49 cases; refractory anaemia with excess of blasts (RAEB), 56 cases; RAEB in transformation (RAEB-T) 48 cases; and chronic myelomonocytic leukemia (CMML), 14 cases. The following scoring systems were applied: Mufti's 1985, Varela's 1985, Sanz's 1989, Rubio's 1991 and Aul's 1992. The statistical analysis was performed according to Kaplan-Meier actuarial systems and the log-rank test of survival.

RESULTS

(1) Three groups (A, B and C) can be defined by Mufti's system, with median survival of 54.0, 16.0 and 8.5 months, respectively. The majority of cases (138) were included in group B. Group A did not reach 25 per cent of actuarial survival probability, whereas groups B and C did at 31.1 and 12.2 months, respectively. With regard to the morphologic subtypes, RA and RSA were included in groups A and B, and RAEB, RAEB-T and CMML pertained mostly to group C. Sixty-six cases (33.6 per cent) developed into acute leukemia (AL) corresponding to those last groups. (2) The three groups defined by Varela's system (0-1, 2-5 and 6 or more) had median survival of 91.8, 24 and 13 months, respectively. As in the former system, group 0-1 did not reach 25 per cent actuarial probability, this appearing at 60 and 20 months, respectively, in groups 2-5 and > 6. The distribution of the cytological varieties, RA and RSA among the groups is heterogenous although they were more common within the cases included in groups 0-1. All cases developing AL were included in the groups 2-5 and > 6. (3) The three groups of the system proposed by Sanz (0-1, 2-3 and 4-5) had median survival of, 55.3, 15 and 12.6 months respectively. As in the preceding cases, group 0-1 did not reach the 25 per cent actuarial probability, while this figure appeared at 28.2 months for group 2-3 and at 19.3 months for group 4-5. RA and RSA varieties were included chiefly in group 0-1, while RAEB and RAEB-T appear mostly in groups 2-3 and 4-5. The distribution of the cases and the evolution of AL was heterogeneous according to this system, although they predominate in groups 2-3 and 4-5. (4) Using the Aul's system, three groups A, B and C were defined. The median survival time was 14 months for group C and 24 months for group B. For group A, the median survival was not reached. RA and RSA were exclusive for group A, while RAEB and RAEB-T varieties were outstanding in group C. Regarding the evolution to leukemia the differences observed had no statistical relevance. (5) Three prognostic groups were defined by Rubio's system (namely 0-2.5, 3-5.5, > or = 6) with median survival of 53.3, 16.8 and 10.5 months, respectively. A striking difference was seen when studying the cumulated survival observed, in each of the three percentages considered, between the groups. The different cytological varieties were reasonably distributed with higher incidence of RA and RSA in group I and RAEB, RAEB-T and CMML in group III. This system offers statistical significance when comparing RA with RSA, RAEB with RAEB-T and, obviously RA+RSA with RAEB+RAEB-T+CMML. The evolution into AL also showed statistical significance with respect to the three groups.

摘要

目的

评估应用于骨髓增生异常综合征(MDS)且具有预测趋势的五种评分系统的预后意义。

患者与方法

本研究纳入了226例根据FAB标准诊断的MDS患者,于1975年1月至1992年4月在我科进行随访。发现以下MDS亚型:难治性贫血(RA)59例;难治性铁粒幼细胞贫血(RSA)49例;难治性贫血伴原始细胞增多(RAEB)56例;转化中的RAEB(RAEB-T)48例;以及慢性粒-单核细胞白血病(CMML)14例。应用了以下评分系统:穆夫蒂1985年的、巴雷拉1985年的、桑斯1989年的、鲁维奥1991年的和奥尔1992年的。根据Kaplan-Meier精算系统和生存对数秩检验进行统计分析。

结果

(1)穆夫蒂系统可将患者分为三组(A、B和C),中位生存期分别为54.0个月、16.0个月和8.5个月。大多数病例(138例)归入B组。A组未达到25%的精算生存概率,而B组和C组分别在31.1个月和12.2个月时达到。就形态学亚型而言,RA和RSA归入A组和B组,而RAEB、RAEB-T和CMML大多属于C组。66例(33.6%)发展为急性白血病(AL),对应于最后几组。(2)巴雷拉系统定义的三组(0 - 1、2 - 5和6及以上)中位生存期分别为91.8个月、24个月和13个月。与前一系统一样,0 - 1组未达到25%的精算概率,2 - 5组和>6组分别在60个月和20个月时达到。细胞类型在各组中的分布,RA和RSA在组间是异质的,尽管它们在0 - 1组的病例中更常见。所有发展为AL的病例都归入2 - 5组和>6组。(3)桑斯提出的系统的三组(0 - 1、2 - 3和4 - 5)中位生存期分别为55.3个月、15个月和12.6个月。与前例一样,0 - 1组未达到25%的精算概率,而2 - 3组在28.2个月时达到,4 - 5组在19.3个月时达到。RA和RSA类型主要归入0 - 1组,而RAEB和RAEB-T大多出现在2 - 3组和4 - 5组。根据该系统,病例分布和AL的演变是异质的,尽管它们在2 - 3组和4 - 5组中占主导。(4)使用奥尔系统,定义了三组A、B和C。C组中位生存时间为14个月,B组为24个月。A组未达到中位生存期。RA和RSA仅属于A组,而RAEB和RAEB-T类型在C组中突出。关于向白血病的演变,观察到的差异无统计学意义。(5)鲁维奥系统定义了三个预后组(即0 - 2.5、3 - 5.5、≥6),中位生存期分别为53.3个月、16.8个月和10.5个月。在研究每组所观察到的累积生存情况时,在考虑的三个百分比中,各组之间存在显著差异。不同细胞类型分布合理,RA和RSA在第I组中发生率较高,RAEB、RAEB-T和CMML在第III组中较高。该系统在比较RA与RSA、RAEB与RAEB-T以及显然RA + RSA与RAEB + RAEB-T + CMML时具有统计学意义。向AL的演变在三组中也显示出统计学意义。

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