Various pathologies in the immune system can lead to complex disorders. As a result, the body will consider its cells to be enemy, as well as fight against them. Autoimmune anemia is considered a rare disease in which antibodies to their red blood cells are formed. The consequences of this phenomenon are serious, since disturbances in the circulatory system affect the functioning of the whole organism.
The concept
Autoimmune anemia is a disease characterized by severe destruction of healthy red blood cells due to the aggressive effects of antibodies on them. These antibodies are produced by the body. This manifests itself in the form of pallor of the skin, enlargement of the liver and spleen, pain in the lower back and abdomen, shortness of breath and other symptoms. To identify the ailment, laboratory research is needed. The treatment will be conservative, but sometimes an operation to remove the spleen is needed.
Autoimmune anemia is observed infrequently. This disease appears in 1 person out of 70-80 thousand. It is usually detected in women. Autoimmune anemia occurs in children and adults. Diagnosis is usually straightforward. Using routine blood tests, doctors can make a diagnosis. Recovery occurs in no more than 50% of cases. But with glucocorticosteroid treatment, an improvement in health is observed in 85-90% of cases.
Code
In the ICD, autoimmune anemia is indicated by code D59. The ailment of this species is associated with the appearance of antibodies to its red blood cells. The disease is acquired and hereditary. The destruction of red blood cells can be intracellular and intravascular.
Autoimmune hemolytic anemia (in the ICD-10 code, see above) is an ailment that should be identified as soon as possible. Often there are complex names of the disease. Autoimmune hemolytic anemia with incomplete thermal agglutins is a term used for a common form of anemia.
Causes
This anemia is idiopathic (primary) and symptomatic (secondary). If the cause of the destruction of red blood cells can be identified, then this is secondary anemia. When an etiological factor is not identified, then anemia will be idiopathic.
Autoimmune hemolytic anemia develops:
- from lymphoblastic leukemia;
- exposure to radiation;
- malignant tumor;
- connective tissue diseases;
- past infections;
- autoimmune ailments not associated with damage to the hematopoietic system;
- type 1 diabetes mellitus;
- antibiotic treatment;
- immunodeficiency conditions.
The most common is the thermal form of anemia, when the body’s internal temperature is normal, and class G immunoglobulins, components C3 and C4, are located on red blood cells. Red blood cells are destroyed only in the spleen with macrophages.
A cold form of the ailment may appear from an unknown cause. It also develops from infection, hypothermia, lymphoproliferative ailments. In the latter case, people from 60 years old get sick. Pathology in the body, in which red blood cells are destroyed, manifests itself after lowering the temperature in the peripheral vessels to 32 degrees. Cold autoagglutinins are M. immunoglobulins.
Hemolysis, which is observed in the spleen, is often very severe. Often the patient cannot be saved. The course of the disease from the infection is usually acute. If the violation appeared from an unknown cause, it will be chronic.
Rarely, paroxysmal cold anemia appears. Hemolysis appears from exposure to cold. Danger arises even when taking cold drinks and washing hands in cool water. Often this anemia appears with syphilis. The severity of the disease can be from time to time. Sometimes an incurable pathology develops, leading to death.
Symptoms
Autoimmune anemia in children and adults is manifested in the form of 2 syndromes: anemic and hemolytic. The first syndrome can be identified:
- pallor of the skin and mucous membranes;
- dizziness;
- frequent nausea
- palpitations;
- weaknesses;
- fatigue.
Autoimmune hemolytic anemia is detected:
- on light yellow or dark yellow skin;
- an increase in the size of the spleen;
- brown urine;
- the appearance of DIC.
Acute anemia usually occurs with an infection of the body. Therefore, in addition to the symptoms of erythrocyte destruction, there are signs of a major ailment.
Cold anemia has a chronic course. Under the influence of low temperatures, fingers and toes, ears, face turn pale. Ulcers and gangrene may appear. Often patients reveal a cold urticaria. Skin lesions remain for a long time.
Thermal anemia has a chronic course. Pathology exacerbates with an increase in temperature, which usually manifests itself in viral and bacterial infections. Symptom is the color of urine in black.
Acute anemia manifests itself in the form of fever, chills, headaches, dizziness. Shortness of breath, abdominal and lower back pain also occur. The skin becomes pale, may be yellow, subcutaneous hemorrhage occurs on the legs. In addition to the spleen, the size of the liver increases.
With a chronic illness, a person's health is satisfactory. Violation can be detected by an increase in the spleen in size and periodically manifested jaundice. After bouts of remission, exacerbations occur.
Diagnostics
To make an accurate diagnosis, you need not only an external examination of the patient. It is necessary to complete the diagnosis of autoimmune hemolytic anemia. In addition to collecting an anamnesis, you need to donate blood. Her analysis shows an increase in ESR, revealed reticulocytosis, normo- or hypochromic anemia, an increase in bilirubin in the blood. And the level of hemoglobin and red blood cells decreases.
Urinalysis is required for analysis. It reveals protein, an excess of hemoglobin and urobilin. Another patient is sent for an ultrasound of the internal organs with a study of the liver and spleen. If the information received is not enough, then bone marrow sampling is required, for which its puncture is performed. After examination of the material, hyperplasia of the brain is detected, which is performed due to the activation of erythropoiesis.
Trepanobiopsy is a procedure that is performed for the same purpose as bone marrow puncture. But it is more difficult for patients to tolerate, so it is used less often.
A direct Coombs test for autoimmune anemia will be positive. But when receiving negative test results, one should not exclude the ailment. This often appears during treatment with hormonal drugs or with very intense hemolysis. Enzyme-linked immunosorbent assay will help to identify the class and type of immunoglobulins that are involved in the autoimmune reaction.
Treatment features
The treatment of autoimmune anemia is usually long and does not always lead to absolute recovery. First you need to determine the reasons that led to the fact that because of what the body began to destroy its red blood cells. If the etiological factor was determined, then it must be eliminated.
If the cause is not established, then treatment of autoimmune hemolytic anemia is performed by means of the glucocorticosteroid group. Often prescribed "prednisone." If the course of the disease is severe and the hemoglobin level drops to 50 g / l, then a red blood cell transfusion is required.
Autoimmune hemolytic anemia is treated with blood detoxification to remove the breakdown products of red blood cells and improve well-being. Plasmapheresis can reduce the level of antibodies circulating in the bloodstream. Symptomatic treatment is necessarily performed. Indirect anticoagulants are prescribed to protect against the development of DIC. It will be possible to maintain a blood-forming system by administering vitamin B12 and folic acid.
If it turned out to cure the disease, then this is the end of the treatment. If after some time the disease reappears, surgery to remove the spleen is required. This will help prevent hemolytic crises in the future, since it is in the spleen that red blood cells accumulate. The procedure often leads to absolute recovery. According to clinical recommendations, autoimmune hemolytic anemia in rare cases is treated with immunosuppressive therapy if splenectomy does not bring positive results.
Use of medicines
Medicines can be used as fixed assets or auxiliary. The most effective include the following:
- "Prednisolone" is a glucocorticoid hormone. The tool inhibits immune processes. So, the aggression of the immune system to red blood cells is reduced. A day will take 1 mg / kg intravenously, drip. With severe hemolysis, the dose rises to 15 mg / day. When hemolytic crisis is passed, the dose is reduced. Treatment is performed until the normalization of hemoglobin and red blood cells. Then the dosage is gradually reduced by 5 mg every 2-3 days until the withdrawal of the drug.
- Heparin is a short-acting direct anticoagulant. The drug is prescribed to protect against DIC, the risk of which increases with a sharp decrease in the number of red blood cells circulating in the blood. Every 6 hours, 2500-5000 IU is administered subcutaneously under the control of a coagulogram.
- "Nadroparin" is a direct long-acting anticoagulant. Indications are the same as those of "Heparin." 0.3 ml / day is administered subcutaneously.
- Pentoxifylline is an antiplatelet agent, therefore it is used to reduce the risk of DIC. Another drug is considered a peripheral vasodilator, which improves blood circulation to peripheral tissues and brain tissues. Inside, 400-600 mg / day is taken in 2-3 doses. Treatment should be performed 1-3 months.
- Folic acid is a vitamin that takes part in various body processes, including the formation of red blood cells. The initial dosage is 1 mg per day. Increasing the dose is allowed with insufficient therapeutic effect. The maximum dose is 5 mg.
- Vitamin B12 is a substance involved in the formation of mature red blood cells. With its shortage, the size of the red blood cell increases, and its plastic properties decrease, which reduces the duration of its existence. 100-200 micrograms are taken orally or intramuscularly per day.
- "Ranitidine" is an H2 antihistamine that reduces the production of hydrochloric acid by the stomach. These measures are required to compensate for the side effect of prednisone on the gastric mucosa. Inside, 150 mg is taken twice a day.
- Potassium chloride is an agent prescribed to compensate for the loss of potassium ions by the body. Take 1 g three times a day.
- "Cyclosporin A" is an immunosuppressant that is taken with insufficient action of glucocorticoids. 3 mg / kg intravenously, drip is prescribed per day.
- "Azathioprine" is an immunosuppressant. 100-200 mg are taken per day for 2-3 weeks.
- Cyclophosphamide is an immunosuppressant. 100-200 mg are taken per day.
- Vincristine is an immunosuppressant. 1-2 mg is used per week.
Each drug has its own indications, contraindications, rules of administration, so first you need to familiarize yourself with the instructions for use. All the funds indicated in the article can be taken only if they have been prescribed by a doctor.
Splenectomy
This procedure is considered forced, it eliminates intracellular hemolysis, reducing the manifestation of the disease. Splenectomy - an operation to remove the spleen - is performed with the 1st exacerbation of the disease after drug therapy. The operation may not be carried out with contraindications from other organs and systems. The effect of the procedure is great and provides absolute recovery, according to various sources, in 74-85% of cases.
Splenectomy is performed in the operating room using intravenous analgesia. The patient is placed in a supine position or lying on the right side. After removal of the spleen, an audit of the abdominal cavity is required for the presence of an additional spleen. If identified, they are removed. This anomaly is rare, but ignorance of this fact leads to diagnostic errors, since after removal there will be a remission of the disease. The decision to perform the operation must be made by the doctor. After this, compliance with all the requirements of a specialist is required, since they will allow you to quickly recover.
Prevention
According to clinical recommendations, autoimmune anemia can be prevented. This requires directing efforts to prevent people from dangerous viruses that can lead to illness.
If the disease has already appeared, then it is necessary to minimize the effect on the body of those factors that can lead to exacerbation. For example, it is important to prevent high and low temperatures. It will not work to protect against idiopathic anemia, because its causes are unknown.
If at least once there was autoimmune anemia, then the next 2 years, blood donation is required for a general analysis. This should be done at intervals of 3 months. Any signs of the disease may indicate a developing ailment. In this case, you need to see a doctor.
Forecast
What is the prognosis of autoimmune hemolytic anemia? It all depends on the degree of the disease. Idiopathic anemia is more difficult to treat. Absolute recovery after a hormonal course will be achieved no more than 10% of patients.
But the removal of the spleen increases the number of recovered persons to 80%. Immunosuppressive therapy is difficult to tolerate, this treatment adversely affects the immune system and leads to complications. The success of therapy depends on the factor that caused the anemia.