Other sideroblastic anaemias - what does this mean
Other sideroblastic anaemias are a group of anaemias caused by an abnormality in the production of red blood cells resulting from a defect in the metabolism of iron. this defect causes the body to produce red blood cells with an abnormal amount of iron, which cannot carry oxygen efficiently, leading to anaemia.
What happens during the disease - other sideroblastic anaemias
Other sideroblastic anaemias are a group of inherited or acquired anaemias characterised by the presence of ringed sideroblasts in the bone marrow. these anaemias are caused by a defect in the haem biosynthesis pathway resulting in impaired production of haemoglobin and hence anaemia. this is caused by a defect in the enzymes involved in the haem biosynthesis pathway, such as alas2, leading to an accumulation of iron in the mitochondria of the red blood cells. this leads to the formation of ringed sideroblasts, which are immature red blood cells containing iron-containing granules.
Treatment and Medical Assistance
Main goal: Treat the underlying cause of the other sideroblastic anaemias.
- Assess for any underlying causes such as medications, infection, or malignancy.
- Evaluate for any nutritional deficiencies and provide appropriate supplementation.
- Administer drugs that inhibit the production of erythropoietin, such as phenylbutazone.
- Administer medications to increase the production of red blood cells, such as vitamin B6.
- Administer drugs to reduce the production of red blood cells, such as hydroxyurea.
- Perform a bone marrow transplant.
- Provide supportive care, such as transfusions and iron supplementation.
13 Days of Hospitalization Required
Average Time for Outpatient Care Not Established
Other sideroblastic anaemias - Prevention
Other sideroblastic anaemias can be prevented by avoiding alcohol consumption, maintaining a healthy diet rich in iron and vitamin b12, and avoiding exposure to toxins such as lead. additionally, regular medical check-ups can help to identify any underlying causes and provide early intervention.