[关于铁母细胞性贫血的新观点]。

Tohru Fujiwara
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引用次数: 0

摘要

铁母细胞性贫血(SAs)代表了一组不同的先天性和获得性疾病,其特征是贫血和骨髓中环状铁母细胞的存在。先天性铁母细胞性贫血(CSA)由基因突变引起,破坏线粒体内的血红素和铁代谢。CSA最常见的形式是x -连锁铁母细胞性贫血(XLSA),由红细胞特异性氨基乙酰化合成酶2 (ALAS2)基因突变引起,ALAS2是红细胞血红素生物合成途径的关键酶。另一方面,获得性SA最常见的形式是环形铁母细胞骨髓增生异常综合征(MDS-RS)。本文综述了目前对sa病理生理的理解和新兴观点,特别关注XLSA和MDS-RS。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Emerging perspectives on sideroblastic anemia].

Sideroblastic anemias (SAs) represent a diverse group of congenital and acquired disorders, characterized by anemia and the presence of ring sideroblasts in the bone marrow. Congenital sideroblastic anemia (CSA) arises from genetic mutations that disrupt heme and iron metabolism within mitochondria. The most common form of CSA is X-linked sideroblastic anemia (XLSA), caused by mutations in the erythroid-specific aminolevulinate synthase 2 (ALAS2) gene, a key enzyme in the heme biosynthesis pathway in erythroid cells. On the other hand, the most common form of acquired SA is myelodysplastic syndrome with ring sideroblasts (MDS-RS). The review explores the current understanding and emerging perspectives on the pathophysiology of SAs, with a particular focus on XLSA and MDS-RS.

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