refractory anemia with ring sideroblasts


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  • Clinical

    • anemia, may have iron overload in liver, spleen
    • may have cytopenia in other lineages
    • 3-11% of MDS
    • median age 60-73
    • 1-2% develop AML

  • Morphology

    • blood
      • anemia
      • normochromic, +/- hypochromic population
      • normo to macrocytic
      • lack blasts
    • marrow
      • normo to hypercellular with increased erythroid precursors
      • erythroid dysplasia 10%
        • nuclear lobation
        • megaloblastoid
      • ring sideroblasts
        • 15% of erythroid precursors
        • 5+ iron granules encircling at least 1/3 of nucleus
      • hemosiderin laden macrophages
      • other lineages normal (< 10% dysplasia)
      • myeloblast < 5%

  • Molecular

    • 5-20% have chromosomal abnormality

  • Ddx

    • congenital sideroblastic anemia (has decreased protoporphyrin IX)
    • other MDS with ring sideroblasts
    • toxic (EtOH, lead, benzene), drugs (INH), zinc
    • copper deficiency
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