Ring sideroblasts and iron metabolism

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  • FUJIWARA Tohru
    Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine Laboratory Diagnostics, Tohoku University Hospital

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  • 環状鉄芽球と鉄代謝
  • カンジョウ テツガキュウ ト テツ タイシャ

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Abstract

<p>Ring sideroblasts show abnormal mitochondrial iron accumulation, and their emergence in the bone marrow is a characteristic of sideroblastic anemias (SAs). SAs are a group of heterogeneous congenital and acquired disorders. Congenital SA is a rare disease caused by gene mutations involved in heme biosynthesis, iron-sulfur cluster biosynthesis, and mitochondrial protein synthesis. SAs can also occur after exposure to certain drugs or alcohol and due to copper deficiency (secondary SA). Furthermore, SAs are associated with myelodysplastic syndrome (idiopathic SA), strongly correlating with specific somatic mutations in splicing factor 3b subunit 1 (SF3B1), which is involved in the RNA splicing machinery. Recent reports have indicated that common defects in iron/heme metabolism underlie in the mechanisms of ring sideroblast formation in congenital and acquired SAs. Current understanding of SA pathophysiology, including the mechanisms of ring sideroblast formation, is discussed in this review.</p>

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  • Rinsho Ketsueki

    Rinsho Ketsueki 61 (7), 770-778, 2020

    The Japanese Society of Hematology

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