Sideroblastic anemia natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Nazia Fuad M.D.
Overview
Natural History
- Majority of patients of sideroblastic anemia at the time of diagnosis shows erythroid abnormalities and ineffective erythropoiesis,
- Granulocytic and megakaryocytic cell lines involvement is also common.
- In the initial stages bone marrow reveal erythroid expansion with ineffective erythropoiesis
- Progression to bone marrow failure occurs in the course of the disease
- The next phase in natural history of sideroblastic anemia is iron overload and evolution to nonlymphocytic leukemia
- The most common causes of death are related to complications of iron overload and evolution into ANLL..[1]
Complications
Common complications of sideroblastic anemia include
- Secondry hemochromatosis
- Thrombocytopenia
- Growth retardation
- Blindness
- Deafness
- Ineffective erythropoiesis
Prognosis
- Depending on the type of sideroblastic anemia the prognosis may vary. However, the prognosis is generally regarded as good[1].
- Sideroblastic anemia secondry to drugs or alcohol as underlying cause is associated with the most favorable prognosis.
- (5-10%) of Severe refractory sideroblastic anemias associated with MDS undergo leukemic transformation.
- AML markedly reduce life expectancy
- Patients who do not need blood transfusions are likely to be long-term survivors.
- The transfusion dependent are at risk of death from the complications of secondary hemochromatosis.
References
- ↑ 1.0 1.1 Cazzola M, Barosi G, Gobbi PG, Invernizzi R, Riccardi A, Ascari E (February 1988). "Natural history of idiopathic refractory sideroblastic anemia". Blood. 71 (2): 305–12. PMID 3337899.