Sideroblastic anemia natural history, complications and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Sideroblastic anemia}} | {{Sideroblastic anemia}} | ||
{{CMG}} {{AE}} | {{CMG}} {{AE}} {{N.F}} | ||
==Overview== | ==Overview== | ||
==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 | * [[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..<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> | * 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== | ||
Common complications of sideroblastic anemia include | Common complications of sideroblastic anemia include | ||
* Secondry hemochromatosis | * [[Secondry hemochromatosis]] | ||
* Thrombocytopenia | * [[Thrombocytopenia]] | ||
* Growth retardation | * [[Growth retardation]] | ||
* Blindness | * [[Blindness]] | ||
* Deafness | * [[Deafness]] | ||
* Ineffective erythropoiesis | * [[Ineffective erythropoiesis]] | ||
==Prognosis== | ==Prognosis== | ||
* Depending on the type of sideroblastic anemia the prognosis may vary. However, the prognosis is generally regarded as good<ref name="pmid3337899" />. | * Depending on the type of sideroblastic anemia the prognosis may vary. However, the [[prognosis]] is generally regarded as good<ref name="pmid3337899" />. | ||
* Sideroblastic anemia secondry to drugs or alcohol as underlying cause is associated with the most favorable prognosis. | * 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. | * (5-10%) of Severe refractory sideroblastic anemias associated with MDS undergo leukemic transformation. | ||
* AML markedly reduce life expectancy | * AML markedly reduce life expectancy | ||
* Patients who do not need blood transfusions are likely to be long-term survivors. | * 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 | * The transfusion dependent are at risk of death from the complications of secondary [[hemochromatosis]]. | ||
==References== | ==References== |
Revision as of 16:43, 7 August 2018
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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.