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骨髓增生异常综合征风险组的检测。一项多中心研究。

Detection of risk groups in myelodysplastic syndromes. A multicenter study.

作者信息

Belli Carolina, Acevedo Susana, Bengio Raquel, Arrossagaray Guillermo, Watman Nora, Rossi Norma, García Juan, Flores Gabriela, Goldztein Sofía, Larripa Irene

机构信息

Instituto de Investigaciones Hematológicas Martino R. Castex, Academia Nacional de Medicina, Pacheco de Melo 3081, CP 1425, Capital Federal, Argentina.

出版信息

Haematologica. 2002 Jan;87(1):9-16.

Abstract

BACKGROUND AND OBJECTIVES

Myelodysplastic syndromes (MDS) comprise a group of heterogeneous hematologic disorders with risk of leukemic evolution (LE). The French-American-British (FAB) co-operative group classifies them into five morphologic entities and the International Prognostic Scoring System (IPSS) proposes four groups of risk on the basis of clinical and cytogenetic variables. The aim of this study was to evaluate the application of the IPSS in our Argentine population, to test the prognostic value of its variables and to determine whether this score helps to associate prognostic subgroups of risk into FAB subtypes.

DESIGN AND METHODS

Two hundred and thirty-four patients with primary MDS and a median follow-up of 28 months were evaluated using univariate analyses to determine median survival (SV) and the time to LE. The variables analyzed were FAB classification, IPSS, percentage of myeloblasts, cytogenetic groups of risk and number of cytopenias.

RESULTS

Univariate analyses showed that all variables analyzed were predictive for SV and for LE in our MDS population. Application of the IPSS allowed discrimination into the 4 groups of risk and helped to identify prognostic subclasses among the FAB classification, associating 5%, 15% and 19% of cases with worse prognosis within the FAB classification of refractory anemia (RA), RA with ringed sideroblasts and RA with excess of blasts (RAEB), respectively. The IPSS was not informative for RAEB in transformation cases and would not be applied to patients with chronic myelomonocytic leukemia.

INTERPRETATION AND CONCLUSIONS

This score could be applied to our MDS population, showing no geographic differences. Stratification of FAB patients according to IPSS would be helpful to develop risk-adapted therapeutic strategies.

摘要

背景与目的

骨髓增生异常综合征(MDS)是一组异质性血液系统疾病,存在白血病转化(LE)风险。法美英(FAB)协作组将其分为五个形态学类型,国际预后评分系统(IPSS)根据临床和细胞遗传学变量提出了四组风险。本研究旨在评估IPSS在阿根廷人群中的应用,检验其变量的预后价值,并确定该评分是否有助于将预后风险亚组与FAB亚型相关联。

设计与方法

对234例原发性MDS患者进行评估,中位随访时间为28个月,采用单因素分析确定中位生存期(SV)和白血病转化时间。分析的变量包括FAB分类、IPSS、原始粒细胞百分比、细胞遗传学风险组和血细胞减少症数量。

结果

单因素分析显示,在我们的MDS人群中,所有分析变量对SV和LE均具有预测性。应用IPSS可将患者分为4个风险组,并有助于在FAB分类中识别预后亚组,在难治性贫血(RA)、环形铁粒幼细胞性RA和原始细胞过多的RA(RAEB)的FAB分类中,分别有5%、15%和19%的病例预后较差。IPSS对转化中的RAEB病例无信息价值,不适用于慢性粒单核细胞白血病患者。

解读与结论

该评分可应用于我们的MDS人群,未显示出地域差异。根据IPSS对FAB患者进行分层,将有助于制定风险适应性治疗策略。

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