Sideroblastic anemia natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
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]]. complications of sideroblastic anemia include secondry [[hemochromatosis]], t[[Thrombocytopenia|hrombocytopenia]], [[Growth retardation]], [[Blindness]], [[Ineffective erythropoiesis]], myocardial siderosis, liver cirrhosis and malabsorption. Prognosis depends on the type and cause of sideroblastic anemia. Sideroblastic anemia secondry to drugs or alcohol as underlying cause is associated with the most favorable prognosis. Sideroblastic anemias associated with MDS undergo leukemic transformation in 5-10% cases. The transfusion dependent are at risk of death from the complications of secondary [[hemochromatosis]]. | |||
==Natural History== | ==Natural History== | ||
* Majority of patients of sideroblastic anemia at the time of diagnosis shows [[erythroid]] abnormalities and ineffective [[erythropoiesis]] | * 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. | * [[Granulocytic]] and [[megakaryocytic]] cell lines involvement is also common. | ||
* In the initial stages bone marrow reveal erythroid expansion with ineffective [[erythropoiesis]] | * In the initial stages bone marrow reveal erythroid expansion with ineffective [[erythropoiesis]]. | ||
* Progression to [[bone marrow failure]] occurs in the course of the disease | * 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 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 | * The most common causes of death are related to complications of iron overload and evolution into ANLL.<ref name="pmid3337899">{{cite journal |vauthors=Cazzola M, Barosi G, Gobbi PG, Invernizzi R, Riccardi A, Ascari E |title=Natural history of [[idiopathic]] refractory sideroblastic anemia |journal=Blood |volume=71 |issue=2 |pages=305–12 |date=February 1988 |pmid=3337899 |doi= |url=}}</ref> | ||
==Complications== | ==Complications== | ||
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* [[Deafness]] | * [[Deafness]] | ||
* [[Ineffective erythropoiesis]] | * [[Ineffective erythropoiesis]] | ||
* Myocardial siderosis | |||
* Liver cirrhosis | |||
* Malabsorption | |||
==Prognosis== | ==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
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. complications of sideroblastic anemia include secondry hemochromatosis, thrombocytopenia, Growth retardation, Blindness, Ineffective erythropoiesis, myocardial siderosis, liver cirrhosis and malabsorption. Prognosis depends on the type and cause of sideroblastic anemia. Sideroblastic anemia secondry to drugs or alcohol as underlying cause is associated with the most favorable prognosis. Sideroblastic anemias associated with MDS undergo leukemic transformation in 5-10% cases. The transfusion dependent are at risk of death from the complications of secondary hemochromatosis.
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
- Myocardial siderosis
- Liver cirrhosis
- Malabsorption
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.