骨髓增生异常综合征(MDS)的诊断、预后和最佳治疗方法

J. Rehman, A. Ni, M. Jalil
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摘要

骨髓增生异常综合征(MDS)是一种以造血功能低下和骨髓发育不良为特征的干细胞疾病,在许多情况下会发展为急性髓性白血病。MDS的治疗是可变的,并根据基于国际预后评分系统(IPSS)的风险分类进行应用[2,3]。大约10-20%的骨髓增生异常综合征(MDS)患者存在自身免疫性疾病(AD),这可能具有挑战性。自身免疫被认为是由发育不良的骨髓细胞凋亡增加引起的。最近的证据表明,这两种疾病都以树突状细胞和t细胞异常为特征。AD的表现各不相同,从临床症状如血管炎、狼疮和类风湿性关节炎到实验室异常如血小板减少症、溶血性贫血和自身抗体[4]。AIM和MDS的关联在1982年首次被描述为MDS诊断后一年的AIHA。6随后,多个病例和研究被发表,强调自身免疫与MDS之间的关系。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Myelodysplastic syndrome (MDS), diagnosis, prognosis and the best available treatment
Myelodysplastic syndrome (MDS) is a stem cell disorder characterized by ineffective hematopoiesis and bone marrow dysplasia that, in many cases, progresses to acute myeloid leukemia [1]. Treatment for MDS is variable and applied according to the risk classification based on the International Prognostic Scoring System (IPSS) [2,3]. Approximately 10–20% of patients with myelodysplastic syndrome (MDS) present with autoimmune diseases (AD) which can be challenging to recognize. The autoimmunity is believed to be triggered by the increased apoptosis in the dysplastic bone marrow. Recent evidence suggests that both diseases are characterized by dendritic and T-cell abnormalities. AD presentation varies from clinical syndromes such as vasculitis, lupus and rheumatoid arthritis to laboratory abnormalities such as thrombocytopenia, hemolytic anemia and autoantibodies [4]. The association of AIM and MDS was first described in 1982 as AIHA one year after the diagnosis of MDS. 6 Subsequently, multiple cases and studies have been published emphasizing the relationship between autoimmunity and MDS [5].
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