Sideroblastic anemia is a rarely diagnosed type of anemia. The essence of the disease is defective iron metabolism, which can lead to cirrhosis of the liver, cardiomyopathy or the development of diabetes mellitus. What are the causes and symptoms of sideroblastic anemia? How is this rare type of anemia treated?

Sideroblastic anemiabelongs to the group of anemia associated with disturbances in the production of erythrocytes (red blood cells). In the course of the disease, the bone marrow, instead of producing red blood cells properly, produces sideroblasts, i.e. erythroblasts that contain a greater number of extra-hemoglobin iron grains. As a consequence, there is an excess of this element in the body.

Contents:

  1. Sideroblastic Anemia - Causes
  2. Sideroblastic Anemia - Symptoms
  3. Sideroblastic anemia - diagnosis
  4. Sideroblastic Anemia - Treatment
  5. Diet for sideroblastic anemia

Sideroblastic Anemia - Causes

In the case of congenital disease, the cause of excess iron in the body are mutations of various genes, related to, among others, disorders in heme synthesis, thiamine-dependent disorders. In turn, at the basis of acquired sideroblastic anemia lie:

  • alcohol abuse
  • copper deficiency in the body
  • use of drugs that inhibit the synthesis of pyridoxal phosphate (isoniazid, cycloserine)
  • zinc or lead poisoning.

The coexistence of myelodysplastic syndrome, bone marrow fibrosis and chronic myeloid leukemia may also contribute to the development of the disease.

Sideroblastic Anemia - Symptoms

The picture of the disease consists of typical symptoms of anemia, i.e .:

  • pale skin, mucous membranes and conjunctiva
  • increased weakness and fatigue quickly
  • headaches and dizziness
  • problems with concentration
  • increased heart rate and rapid breathing
  • feeling cold

and symptoms related to excess iron in the body: diabetes mellitus, arrhythmias and neurological disorders. Some patients also experience enlargement of the liver andspleen.

The inborn form of the disease usually shows up in early childhood. The acquired form is most common in people over 50.

Sideroblastic anemia - diagnosis

Disease diagnosis is based on blood tests, bone marrow aspiration or trepanobiopsy, as well as cytogenetic testing.

Blood morphology usually reveals a decrease in hemoglobin levels, an accumulation in erythrocytes of iron, copper, copro- and protoporphyrins, decreased number of platelets and leukocytes. A biopsy, i.e. the collection of bone marrow from the iliac plate, allows for the presence of ring sideroblasts and an increased amount of iron in the marrow cells. In turn, cytogenetic tests reveal chromosomal abnormalities.

Sideroblastic Anemia - Treatment

In the case of genetically determined sideroblastic anemia, vitamin B6 therapy is used. A response to treatment is usually obtained within a few weeks of starting treatment. In patients with a congenital form of the disease, concentrates of red blood cells are transfused regularly and, due to the increased amount of iron in the body, drugs that bind this element are used. In some patients, an autologous bone marrow transplant may be considered.

Important

Diet for sideroblastic anemia

Some products may contribute to the exacerbation of the symptoms of the disease, especially those rich in zinc, i.e. shellfish, liver, hard cheeses, as well as various types of nuts. Therefore, patients should exclude them from the diet, as well as alcohol, which is one of the risk factors for the development of the acquired disease.

Category:

Cardiology