Hypoplastic anemia - blood pathology characterized by decreased production of blood cells in the bone marrow in the absence of cancer. The underlying hypoplasia (underdevelopment) or aplasia of the bone marrow, is responsible for red blood cell formation. This pathology is characterized by severe and the development of dangerous complications such as hemorrhagic syndrome, coma and organ failure.
The annual incidence is about 20 cases per 100,000 people. Men and women suffer equally often. Most often, anemia is diagnosed in people 10-25 years of age and older than 50 years. The basis of this pathology is pancytopenia (lack of leukocytes, platelets and erythrocytes).
Causes
Risk factors for the development of aplastic (aplastic) anemia are:
- Effects on the body of chemicals. It may be industrial poisons, chemical agents, dyes, heavy metals, heavy chemotherapy and other medications (Antineoplastic agents, antibiotics, anti-malarial and anti-seizure drugs, oral hypoglycemic agents, diuretics and gold preparations). Some medications have myelotoxic effects (affecting the bone marrow).
- Autoimmune disorders.
- Exposure to radiation (ionizing radiation).
- Viral diseases. Anemia often occurs after viral hepatitis predominantly parenteral transmission mechanism. The causative agent is in the blood, bone marrow cells, leading to disruption of hematopoiesis. Less commonly, anemia is caused by the flu, mononucleosis, herpes and cytomegalovirus infection.
- Tuberculosis.
- Poisoning.
- Injury.
- Effects on body vibration and high-frequency current.
- Mycoses.
- Error in the diet. With a shortage of substances involved in hematopoiesis, hematopoiesis may be disturbed by the process.
- Systemic diseases (rheumatoid arthritis, systemic lupus erythematosus).
- Endocrine pathology (thyroid disease, diabetes, ovarian pathology and adrenal glands).
- Genetic mutations.
- Congenital malformations.
- Family history.
Classification
Hypoplastic anemia are congenital and acquired. In the first case, a violation of hematopoiesis and a lack of blood cells are found already at birth. Allocate Fanconi anemia (combined with anomalies of bodies), anemia Oestrich-Dameshek (characterized by a general violation of hematopoiesis branches without any congenital disorders) and Dayemonda Black fan-syndrome (reduced output only red blood cells).
Acquired anemia occur during the life mainly in adults and adolescents. Are acute (sudden onset different), subacute (continues up to six months) and chronic (lasting more than six months) forms of this pathology.
Symptoms and Diagnosis
Signs of hypoplastic anemia are:
- General weakness.
- Malaise.
- Dizziness. The reason for this symptom - oxygen deprivation of the brain due to decreased number of red blood cells.
- Fast fatiguability.
- performance decline.
- Pale skin and mucous membranes.
- Noise in ears.
- Shortness of breath that occurs during physical exertion.
- Tingling in the chest.
- Fainting.
- Flies before his eyes.
- Daytime sleepiness.
- Sleep disturbance.
- Chest pain.
- Bleeding and hemorrhage.
- Petechial rash.
- Bleeding gums.
- Signs of oral mucosa.
- Menorrhagia (heavy monthly).
- Nosebleeds.
Congenital hypoplastic anemia may be associated with microcephaly (decreasing brain size) the slow growth of the child, in violation of skin pigmentation, hearing loss and congenital anomalies of development of various authorities.
The diagnosis is based on history puts hematologist for diagnosis is carried out:
- Physical examination. Valuable diagnostic features of this form of anemia are no lymphadenopathy and normal spleen and liver dimensions.
- Examination of the skin and mucous membranes. Often this disease detected ulcerous-necrotic changes in the mucosa.
- General blood analysis. Detects decrease of leukocytes, erythrocytes and tromtsbotsitov or only some of erythrocytes. At low levels of hemoglobin color index is within normal limits. Neutrophil count decreased.
- General urine analysis. In the urine of patients are often found erythrocytes (cause a hemorrhagic syndrome), bacteria (in case of infection of joining) and a large number of leukocytes.
- Blood chemistry.
- Needling followed study punctate. A decrease in the number of megakaryocytes and myelokaryocytes.
- Biopsy. Reveals a decrease in the volume of functional tissue.
- Electrocardiography.
- Other instrumental examinations (FEGDS, sigmoidoscopy, colonoscopy, salpingography) held in bleeding.
Differential diagnosis with other anemia (iron deficiency, post-hemorrhagic, thalassemia, hemolytic) and blood cancer.
Treatment and prognosis
Aplastic anemia requiring hospitalization in the hematology department, and comprehensive treatment. It includes:
- Complete patient isolation.
- Ensuring complete aseptic and antiseptic.
- bone marrow transplantation.
- Stem cell transplantation. Most often, they are taken from the bone marrow of the iliac bone. Apply donor hematopoietic stem cells. The best results are observed for the transplant at a young age. It refers to the experimental treatments.
- The use of immunosuppressants (cyclosporin Sandoz, Ekorala, Panimuna Bioran). It is required in case of failure of the bone marrow transplant. Also effective anti-thymocyte immunoglobulin.
- The use of corticosteroids. These drugs affect leukocyte production.
- The use of anabolic steroids.
- Transfusion (transfusion of blood components).
- Plasmapheresis (blood purification by means of a special device).
- Using hemostatic. These drugs are required for aplastic anemia, accompanied by bleeding of various etiologies.
- The use of antimicrobial agents.
- Splenectomy (removal of the spleen). This body contributes to the destruction of old red blood cells.
- The use of erythropoiesis stimulants. Appointed by drugs such as erythropoietin, Recormon, Epostim, Eritrea and epoetin beta. Erythropoietin is used in the form of a solution for injectable administration. The drug increases the production of red blood cells and reticulocytes and hemoglobin enhances education. Erythropoietin is contraindicated in heart attack and stroke a history of intolerance to the preparation, form unstable angina, high blood pressure and thrombo-embolism.
In the absence of proper treatment of the probability of death reaches 90%. The forecast is largely determined by the degree of shortage of blood cells, the presence of complications, patient's age, comorbidities and disease progression rates. Bone marrow transplantation can achieve sustained remission in most patients.
prevention
Methods of prevention hypoplastic anemia are:
- prevention of infectious diseases;
- the exclusion of external factors (toxic substances, radiation, trauma);
- maintaining a healthy lifestyle;
- nutrition;
- timely treatment system and other disorders.
Specific preventive maintenance is not developed.