This is a violation of hematopoiesis occurs much less frequently than other forms of anemia. However sideroblastic anemia fraught with serious complications, including the destruction of the internal organs and nervous system until the development of mental retardation. Prevent such dangerous consequences allow early detection and treatment of disease.
classification of diseases
The main feature of this form of anemia - iron deficiency in the composition of red blood cells when excessive amounts in the body. Such violation arises from the fact that bone marrow cells lose the ability to make full use of this trace element in the synthesis of hemoglobin. Unused iron gradually accumulates in the liver and spleen. Hemoglobin-depleted red blood cells are not able to provide the cells with oxygen.
When the diagnosis is necessary to find out the causes, the nature and severity of disease. On this depends its kind. Sideroblastic (sideroblasticheskaya, sideroahrestical) anemia can be:
- Purchased.
- Hereditary.
Acquired pathology - most often the result of exposure to toxic substances in the body, a long stay in cold conditions. After elimination of the chemical or thermal stimulus in most clinical cases, the plasma iron content is normalized independently. Harder, if a violation of its generation caused by autoimmune diseases or cancer. Every tenth patient develops leukemia.
Hereditary sideroblastic anemia transmitted to the fetus through the defective maternal chromosome. Due to genetic mutation in hemoglobin synthesis slowed down. In uncomplicated course of the disease and healthy children are born to mothers, and children with severe disease. The disease manifests itself most often in its infancy, but sometimes much later. Classification distinguishes congenital anomalies that are responsive to pyridoxine. Isolated group comprise hereditary disease that may not be correct vitamin B6.
Causes
For the production of hemoglobin requires the following biochemical triad:
- Iron.
- Protoporphyrin.
- Protein.
Sideroblastic form of the disease develops as a result of protoporphyrin deficit. This pigment is a precursor of heme - porphyrin compounds with iron, which is further converted to hemoglobin.
The main causes of acquired and inherited anemia sideroahrestical:
- long-term use of alcohol;
- intoxication compounds of zinc or lead;
- copper deficiency in the body;
- prolonged therapy with certain hormonal drugs, antibiotics, antituberculosis agents (e.g., chloramphenicol, cyclosporine, isoniazid);
- autoimmune diseases (rheumatoid arthritis, type I diabetes, multiple sclerosis, Hashimoto's thyroiditis, celiac disease, etc.);
- malignant tumor;
- advanced age (over 50-55 years);
- genetic predisposition.
symptoms
For the disease characterized by the presence of anemia. Its most common manifestations are:
- dizziness;
- shortness of breath, even with a small physical exertion;
- fast fatiguability;
- prostration;
- headache;
- increased hair loss, brittle nails;
- pain in the heart.
Sideroblasticheskaya hereditary anemia in the majority of clinical cases seen in infancy. The child has the following symptoms:
- confusion, poor memory;
- frequent headaches;
- pale skin;
- muscle weakness;
- fast fatiguability;
- increase in liver, spleen.
In adults, this form of disease can manifest these symptoms:
- earthy grayish or yellowish tint of the skin;
- rapid fatigue at elevated physical activity;
- increase in liver, spleen;
- an increased concentration of glucose in the blood;
- tachycardia, cardiac pain;
- pulmonary insufficiency;
- obesity, weakening of potency, increase in voice.
The intensity of the symptoms of sideroblastic anemia depends on its shape and age of the patient. Manifestations of disease may be minimal, light, especially in the early stages of the disease. Pronounced, severe syndrome is observed in the elderly with gemosideroze - deposition of iron compounds in the tissue cells.
Diagnostics
A blood test reveals form and stage of development of the disease, for which typical figures following laboratory tests:
- increased concentration of serum iron;
- reducing the number of reticulocytes (protoeritrotsitov);
- presence of abnormal erythrocyte (basophilic granulocytes);
- drop in platelet levels, changes in leukocyte counts (in severe liver damage);
- a reduced color index (less than 0.8), and others.
If the content of the erythrocyte protoporphyrin reduced, such indication may indicate a hereditary disease. Detection of blood lead compounds sideroahrestical proves the existence of acquired anemia. To clarify the diagnosis is assigned to X-rays. If the picture of the knee clearly visible areas of calcified bone and a line lead deposits, this indicates chronic intoxication.
Additional methods of research:
- urinalysis, desferalovy test (determination of the presence of iron in urine);
- coprogram;
- US kidney, heart, abdominal organs;
- ECG;
- myelogram biopsy and bone marrow (if necessary).
Treatment and prevention of disease
Specialized professionals, which is engaged in therapy sideroblastic anemia is a hematologist. However, to clarify treatment strategies often require consultation with an endocrinologist, cardiologist, rheumatologist or dermatologist.
In identifying the acquired form of pathology is necessary to arrest the symptoms of the disease and restore blood formula. This is facilitated by:
- elimination of toxic factor provocateur;
- Pyridoxine injection;
- intramuscular or subcutaneous administration to prevent desferal hemosiderosis;
- pentatsina intravenous injection (for lead intoxication);
- transfusion of red blood cells (for severe anemia).
Cure the hereditary form of the disease is impossible. It can only correct symptomatic means able to suppress manifestation of a defective gene. To improve the condition of the patient to help Pyridoxine injection. Synthesis of hemoglobin often activated for 2-3 months under the condition that the patient has no resistance to vitamin B6.
Sometimes the disease is accompanied by megaloblastic bleeding. In this case assigned therapy vitamin B9 (folic acid). In order to avoid the development of hemosiderosis internal organs is strictly prohibited the use of iron supplements.
To avoid the risk of anemia, you should:
- keep track of nutrition, eating beef, liver, legumes, eggs, nuts, dried fruit, rich in iron, vitamins B6, B9, B12;
- take preventive mineral and vitamin complexes in the winter;
- do not abuse alcohol;
- more often to breathe fresh air.
Doctors strongly recommend to check the status of their health every year, as soon as possible to diagnose the pathological processes occurring in the body is hidden. Especially important to promptly treat identified inflammatory diseases of the stomach and intestines.
