Sideroblastic Anemia

Also see Anemia: General Considerations.

Definition AND EtiologyTop

Sideroblastic anemia refers to a heterogeneous group of disorders of heme synthesis characterized by the presence of hypochromic red blood cells and accumulation of iron in the cytoplasm of erythroblasts (sideroblasts). It may be inherited or acquired. Acquired sideroblastic anemia may be further divided into clonal (classified as myelodysplastic syndrome) or metabolic (reversible), caused by copper or pyridoxine deficiency, zinc or lead poisoning, drugs (isoniazid, cycloserine, chloramphenicol), or alcohol dependency.

Clinical FeaturesTop

Clinical features are nonspecific and include general symptoms of anemia (in patients with severe disease) as well as manifestations of the underlying condition. Complete blood count (CBC) and biochemical tests: see Table 1 in Iron Deficiency Anemia. Bone marrow examination reveals increased iron deposition and ring sideroblasts (the diagnostic criterion for myelodysplastic syndrome (MDS) with ring sideroblasts is >10% of ring sideroblasts).

TreatmentTop

Treatment of the underlying condition in acquired reversible cases. In inherited anemia attempt a course of treatment with pyridoxine 50 to 200 mg/d; if this is ineffective, use symptomatic treatment (packed red blood cell transfusions, treatment of iron overload).