Treatment of higher-risk myelodysplastic syndrome

A. Savic, D. Marisavljević, A. Bogdanović
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Abstract

Introduction. The myelodysplastic syndromes are a group of clonal haematopoietic stem cell disorders characterized by cytopenia, dysplasia, ineffective hematopiesis, recurrent genetic abnormalities, and increased risk of developing acute myeloid leukemia. In this paper, we present the review and recommendations for treatment of high risk myelodysplastic syndromes on behalf of the Serbian myelodysplastic syndromes group. Material and Methods. A literature review was conducted using the following bibliographic databases: Google Scholar, MEDLINE and Kobson. The recommendations treatment of high risk myelodysplastic syndromes are based on expert opinion based on review of literature and contemporary recommendations for treatment of high risk for myelodysplastic syndromes. Recommendations. Higher-risk myelodysplastic syndromes should be defined in patients risk group with > 3.5 IPSS-R score. Allo- HSCT is recommended in fit higher-risk patients with IPSS-R > 3.5 as well as in fit lower-risk patients with poor risk features according to EBMT/ELN International expert panel and myelodysplastic syndromes right group. Acute myeloid leukemia like or hypomethylation treatment before Allo-HSCT is indicated in patients with myelodysplastic syndromes with ? 10% of blasts. Azacitidine is recommended in intermediate-2 and high risk IPSS patients who are not eligible for transplantation with minimal number of six cycles to define response. Acute myeloid leukemia like treatment is recommended in fit higher-risk for patients with myelodysplastic syndromes with excess of blasts, good performance status, without substantial comorbidities, and with no poor/very poor cytogenetics/genetics. Conclusion. The treatment of fit higher-risk patients should be based on allo-SCT. In patients who are not candidates for transplant hypomethylation treatment is indicated as well as acute myeloid leukemia like treatment in selected patients.
治疗高危骨髓增生异常综合征
介绍。骨髓增生异常综合征是一组克隆性造血干细胞疾病,其特征是细胞减少、发育不良、造血功能低下、复发性遗传异常和发展为急性髓系白血病的风险增加。在本文中,我们代表塞尔维亚骨髓增生异常综合征组提出了对高危骨髓增生异常综合征治疗的回顾和建议。材料和方法。使用以下书目数据库:Google Scholar、MEDLINE和Kobson进行文献综述。高危骨髓增生异常综合征的治疗建议是基于文献综述和当代高危骨髓增生异常综合征治疗建议的专家意见。建议。高风险骨髓增生异常综合征应在IPSS-R评分> 3.5的患者风险组中定义。根据EBMT/ELN国际专家小组和骨髓增生异常综合征右组,建议在适合IPSS-R > 3.5的高风险患者以及适合低风险但风险特征较差的患者中进行同种异体造血干细胞移植。急性髓系白血病样或低甲基化治疗适用于骨髓增生异常综合征患者。10%的爆炸。阿扎胞苷推荐用于不适合移植的中-2和高风险IPSS患者,至少需要6个周期来确定反应。急性髓性白血病样治疗推荐用于具有骨髓增生异常综合征的高危患者,这些患者具有过多的原细胞,良好的表现状态,没有大量的合并症,并且没有不良/非常差的细胞遗传学/遗传学。结论。适合高危患者的治疗应基于同种异体细胞移植。对于不适合移植的患者,应进行低甲基化治疗,并在选定的患者中进行急性髓系白血病样治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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