Different pathologies in the immune system can lead to complex disorders. As a result, the body will consider its cells as enemy, and also fight them. Autoimmune anemia is considered a rare disease in which antibodies are formed against one's own red blood cells. The consequences of this phenomenon are serious, as disturbances in the functioning of the circulatory system affect the functioning of the whole organism.
Concept
Autoimmune anemia is a disease characterized by severe destruction of he althy 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 pale skin, enlarged liver and spleen, pain in the lower back and abdomen, shortness of breath and other symptoms. To identify the disease, laboratory tests are needed. Treatment will be conservative, but sometimes surgery is needed to remove the spleen.
Autoimmune anemia is rare. This diseaseappears in 1 person out of 70-80 thousand. It is usually found in women. Autoimmune anemia occurs in children and adults. The diagnosis is usually easy. With the help of routine blood tests, doctors can make a diagnosis. Recovery occurs in no more than 50% of cases. But when treated with glucocorticosteroids, an improvement in he alth is observed in 85-90% of cases.
Code
In the ICD, autoimmune anemia is coded D59. The disease of this species is associated with the appearance of antibodies to their red blood cells. The disease is acquired and hereditary. RBC destruction can be intracellular or intravascular.
Autoimmune hemolytic anemia (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 heat agglutins is a term used for a common form of anemia.
Reasons
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 the etiological factor is not identified, then the anemia will be idiopathic.
Autoimmune hemolytic anemia develops:
- from lymphoblastic leukemia;
- exposure to radiation;
- malignant tumor;
- connective tissue diseases;
- past infections;
- autoimmune diseases not related to damage to the hematopoietic system;
- diabetes type 1;
- antibiotic treatment;
- immunodeficiency states.
The most common is the thermal form of anemia, when the internal environment of the body has a normal temperature, and the erythrocytes contain class G immunoglobulins, components C3 and C4. Erythrocytes are destroyed only in the spleen by macrophages.
The cold form of the disease may appear from an unknown cause. It also develops from infection, hypothermia, lymphoproliferative diseases. In the latter case, people from 60 years of age get sick. Pathology in the body, in which red blood cells are destroyed, manifests itself after the temperature in the peripheral vessels drops to 32 degrees. Cold autoagglutinins are immunoglobulins M.
Hemolysis, which is observed in the spleen, is often severe. Often the patient cannot be saved. The course of the disease from infection is usually acute. If the violation appeared from an unknown cause, then it will be chronic.
Rarely, paroxysmal cold anemia occurs. Hemolysis appears from exposure to cold. The danger arises even when taking cold drinks and washing hands in cool water. Often this anemia appears with syphilis. The severity of the disease may vary from case to case. Sometimes an incurable pathology develops, leading to death.
Symptoms
Autoimmune anemia in children and adults manifests itself in the form of 2 syndromes: anemic and hemolytic. You can identify the first syndrome:
- by pale skin and mucous membranes;
- bouts of dizziness;
- frequent nausea;
- strong heartbeat;
- weaknesses;
- fatigue.
Autoimmune hemolytic anemia detected:
- for light yellow or dark yellow skin;
- enlargement of the spleen;
- brown urine;
- appearance of DIC.
Acute anemia usually occurs when the body becomes infected. Therefore, in addition to the symptoms of the destruction of red blood cells, there are signs of the underlying disease.
Cold anemia has a chronic course. Under the influence of low temperatures, the fingers and toes, ears, face turn pale. Ulcers and gangrene may appear. Patients often present with cold urticaria. Skin lesions remain for a long time.
Heat anemia is chronic. Pathology is exacerbated with an increase in temperature, which usually manifests itself in viral and bacterial infections. The symptom is black urine.
Acute anemia manifests itself in the form of fever, chills, headaches, dizziness. There is also shortness of breath, pain in the abdomen and lower back. The skin becomes pale, may be yellow, subcutaneous hemorrhages occur on the legs. In addition to the spleen, the size of the liver increases.
In a chronic illness, a person's he alth is satisfactory. Violation can be identified by an increase in the size of the spleen and intermittent jaundice. After bouts of remission, exacerbations occur.
Diagnosis
To make an accurate diagnosis, you need not onlyexternal examination of the patient. It is necessary to perform a full diagnosis of autoimmune hemolytic anemia. In addition to collecting an anamnesis, you need to donate blood. Its analysis shows an increase in ESR, reticulocytosis, normo- or hypochromic anemia, and an increase in bilirubin in the blood are detected. And the level of hemoglobin and red blood cells goes down.
Urine analysis required. It reveals protein, excess 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 a bone marrow sampling is required, for which its puncture is performed. After examining the material, brain hyperplasia 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 frequently.
Direct Coombs test for autoimmune anemia will be positive. But when you receive negative test results, you should not exclude the disease. This often appears during treatment with hormonal agents or with severe hemolysis. Enzyme immunoassay will help identify the class and type of immunoglobulins that are involved in the autoimmune reaction.
Features of treatment
Treatment for autoimmune anemia is usually lengthy and does not always lead to absolute recovery. First you need to determine the reasons that led to the fact that the body began to destroy its red blood cells. If etiologicalfactor has been determined, then you need to eliminate it.
If the cause is not established, then the treatment of autoimmune hemolytic anemia is performed by means from the group of glucocorticosteroids. Prednisolone is often prescribed. 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 reduces the level of antibodies circulating in the bloodstream. Symptomatic treatment is mandatory. To protect against the development of DIC, indirect anticoagulants are prescribed. It will be possible to support the hematopoietic system by introducing vitamin B12 and folic acid.
If it turned out to cure the disease, then the treatment is over. If after some time the disease reappears, surgery is required to remove the spleen. This will help prevent the occurrence of hemolytic crises in the future, since it is in the spleen that red blood cells accumulate. The procedure often leads to absolute recovery. Clinical guidelines suggest that autoimmune hemolytic anemia is rarely treated with immunosuppressive therapy if splenectomy fails.
Medicine use
Medicines can be used as the main means or auxiliary. To the mosteffective include the following:
- "Prednisolone" is a glucocorticoid hormone. The drug inhibits immune processes. So, the aggression of the immune system to red blood cells is reduced. Per day, 1 mg / kg will be administered intravenously, drip. With severe hemolysis, the dose is increased to 15 mg / day. When the hemolytic crisis is over, the dose is reduced. Treatment is performed until the normalization of hemoglobin and erythrocytes. Then the dosage is gradually reduced by 5 mg every 2-3 days until the drug is discontinued.
- "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 injected subcutaneously under the control of a coagulogram.
- "Nadroparin" - a direct long-acting anticoagulant. The indications are the same as those of Heparin. Subcutaneously injected 0.3 ml/day.
- "Pentoxifylline" is an antiplatelet agent, therefore it is used to reduce the risk of DIC. Another drug is considered a peripheral vasodilator, which improves the blood supply to peripheral tissues and brain tissues. Inside, 400-600 mg / day is taken in 2-3 doses. Treatment should be carried out for 1-3 months.
- Folic acid is a vitamin involved in various body processes, including the formation of red blood cells. The initial dosage is 1 mg per day. An increase in the dose is allowed with insufficient therapeutic effect. The maximum dose is 5mg.
- Vitamin B12 - a substance involved information of a mature erythrocyte. With its shortage, the size of the erythrocyte increases, and its plastic properties decrease, which reduces the duration of its existence. 100-200 mcg is taken orally or intramuscularly per day.
- "Ranitidine" - H2-antihistamine that reduces the production of hydrochloric acid by the stomach. These measures are required to compensate for the side effect of prednisolone on the gastric mucosa. Inside taken 150 mg twice a day.
- Potassium chloride - a remedy prescribed to compensate for the loss of potassium ions in the body. Take 1 g three times a day.
- "Cyclosporin A" - an immunosuppressant, which is taken with insufficient action of glucocorticoids. A day is prescribed 3 mg / kg intravenously, drip.
- Azathioprine is an immunosuppressant. Take 100-200 mg per day for 2-3 weeks.
- "Cyclophosphamide" is an immunosuppressant. 100-200 mg are taken per day.
- Vincristine is an immunosuppressant. 1-2 mg drip per week.
Each drug has its own indications, contraindications, rules of admission, so you first need to read the instructions for use. All the remedies mentioned in the article can only be taken if they have been prescribed by a doctor.
Removal of the spleen
This procedure is considered forced, it allows you to exclude 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 performed 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 under intravenous anesthesia. The patient is placed in the 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. When found, they are removed. This anomaly is rarely observed, but ignorance of such a fact leads to diagnostic errors, since after removal there will be a remission of the disease. The decision to perform the operation should be made by the doctor. After that, compliance with all the specialist's prescriptions is required, as they will allow you to quickly recover.
Prevention
According to clinical guidelines, autoimmune anemia can be prevented. This requires the direction of efforts to prevent a person from dangerous viruses that can lead to illness.
If the disease has already appeared, then it is necessary to minimize the impact on the body of those factors that can lead to an exacerbation. For example, it is important to avoid high and low temperatures. There is no way to protect against idiopathic anemia because its causes are unknown.
If at least once there was autoimmune anemia, then the next 2 years require blood donation for a general analysis. This should be done every 3 months. Any signs of the disease may indicate a developing disease. In this case, a visit to a doctor is required.
Forecast
What is the prognosis for 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 can be achieved no more than 10% of patients.
But removing the spleen increases the number of recovered individuals by up to 80%. Immunosuppressive therapy is difficult to tolerate, this treatment negatively affects the immune system and leads to complications. The success of therapy depends on the factor that caused the anemia.
