Sideroblastic anaemia and treatment

Sideroblastic anaemia also known as refractory anemia with ringed sideroblasts (RARS) is a term used to describe a group of rare blood disorders characterized by the bone marrow's inability to manufacture normal red blood cells. It is caused by the abnormal production of red blood cells, usually as part of myelodysplastic syndrome, which can evolve into hematological malignancies (especially acute myelogenous leukemia). Thus, the body has iron available, but cannot incorporate it into hemoglobin.

Causes

Sideroblastic anaemia is caused due to a failure to completely form heme molecules, whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus of the developing red blood cell.

Some of the secondary causes include:
• Inflammatory conditions e.g. rheumatoid arthritis
• Systemic lupus erythematosus
• Lead poisoning
• Pregnancy
• Chronic alcoholism
• Haemolytic anaemia
• Chronic infections
• Drug toxicity - e.g. chloramphenicol, cycloserine, isoniazid, linezolid, pyrazinamide
• Malabsorption syndromes
• Myxoedema
• Genetic (ALA synthase deficiency (X-linked))
• Nutritional deficiency (pyridoxine or copper deficiency)

Symptoms and Diagnosis

Clinical features are those related to anaemia in general. Symptoms reflect the cytopenias, i.e. anaemia, infection, bruising and haemorrhage. There are no specific signs or symptoms related to sideroblastic anaemia alone.

Diagnosis is made from bone marrow examination demonstrating the presence of ring sideroblasts with a generalized increase in iron stores.

Treatment

Non-Drug treatment

• Treatment is mainly supportive.
• Red cell transfusion is given for symptomatic anaemia.
• Iron chelation with desferrioxamine should be considered after 20-25 units of red cells have been received.
• Avoid alcohol and vitamin C as these increase iron absorption

Drugs

• The use of erythropoietin ± granulocyte colony-stimulating factor has been demonstrated to reduce the need for red cell transfusion in some patients with myelodysplastic syndrome but the studies were small.
• Patients with hereditary sideroblastic anaemia may respond to pyridoxine.
• Cyclosporin A has been shown to give a response rate (i.e. alteration of disease progression or remission) of 62.5% in patients with myelodysplastic syndromes including sideroblastic anaemia.
• New therapies are emerging, approved by the United States FDA in 2004. There are two DNA methyltransferase inhibitors (azacitidine and decitabine) and an immunomodulatory agent (lenalidomide).

Surgical

• Allogenic peripheral stem cell transplantation has been used with success in pyridoxine refractory hereditary sideroblastic anaemia. Few patients are eligible for transplant.

Cost of treatment

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Source: Patient UK

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