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Sideroblastic anemias are iron-utilization anemias that are usually part of a myelodysplastic syndrome, causing a normocytic-normochromic anemia with high RBC distribution width or a microcytic-hypochromic anemia, particularly with increased serum iron and ferritin and transferrin saturation.
Sideroblastic anemias are among the anemias characterized by inadequate marrow utilization of iron for Hb synthesis despite the presence of adequate or increased amounts of iron (iron-utilization anemias). Other iron-utilization anemias include some hemoglobinopathies, primarily thalassemias (see Thalassemias). Sideroblastic anemias are characterized by the presence of polychromatophilic, stippled, targeted RBCs (siderocytes). Sideroblastic anemias are generally part of a myelodysplastic syndrome but may be hereditary or may occur secondary to drugs (eg, chloramphenicol, cycloserine, isoniazid, pyrazinamide) or toxins (including ethanol and lead). Pyridoxine deficiency can lead to sideroblastic anemia. Deficient reticulocyte production, intramedullary death of RBCs, and bone marrow erythroid hyperplasia (and dysplasia) occur. Although hypochromic RBCs are produced, other RBCs may be large, producing normochromic indices; if so, variation in RBC size (dimorphism) usually produces a high RBC distribution width (RDW).
Sideroblastic anemia is suspected in patients with microcytic anemia or a high RDW anemia, particularly with increased serum iron, serum ferritin, and transferrin saturation (see Iron Deficiency Anemia). The peripheral smear shows RBC dimorphism. RBCs may appear stippled. Bone marrow examination is necessary and reveals erythroid hyperplasia. Iron staining reveals the pathognomonic iron-engorged paranuclear mitochondria in developing RBCs (ringed sideroblasts). Other features of myelodysplasia, such as chromosomal abnormalities, are frequently evident. Serum lead is measured if sideroblastic anemia has an unknown cause.
Elimination of a toxin or drug (especially alcohol) can lead to recovery. Rarely, congenital cases respond to pyridoxine 50 mg po tid, but incompletely. Pyridoxine deficiency is corrected by vitamin B 6 supplementation.
Drug NameSelect Trade
pyrazinamideNo US brand name
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