Autoimmune Anemia - Causes, Symptoms And Treatment

2024 Author: Josephine Shorter | [email protected]. Last modified: 2023-12-16 21:43

Autoimmune anemia

Autoimmune anemia is a disease characterized by increased destruction of healthy red blood cells due to the aggressive effect of antibodies on them. These antibodies are produced by the body itself. The pathology is manifested by pallor of the skin, an increase in the liver and spleen in size, pain in the lower back and abdomen, shortness of breath and other symptoms. To detect anemia of an autoimmune nature, laboratory tests will be required. Treatment is conservative, although surgery to remove the spleen is sometimes required.

Autoimmune anemia is not very common. One person out of 70-80 thousand suffers from this disease. More often, autoimmune anemia is diagnosed in women, age does not matter. This type of anemia develops in both children and adults.

As a rule, the diagnosis of anemia of an autoimmune nature is not difficult. Standard blood tests make it possible to make a correct diagnosis in 90% of cases.

Full recovery is observed in no more than 50% of cases. However, an improvement in human well-being against the background of treatment with glucocorticosteroids occurs in 85-90% of cases.

Content:

• Causes and pathogenesis of the development of autoimmune anemia
• Symptoms of autoimmune anemia
• Diagnosis of autoimmune anemia
• Autoimmune anemia treatment
• Prevention and prognosis of the disease

Causes and pathogenesis of the development of autoimmune anemia

Autoimmune anemia can be idiopathic (primary) or symptomatic (secondary). If the cause of the destruction of red blood cells can be established, then they speak of secondary anemia. When the etiological factor remains unclear, the anemia is called idiopathic.

The reasons for the development of autoimmune anemia (secondary) are:

• Acute or chronic lymphoblastic leukemia.
• Exposure to radiation on the human body.
• The presence of a malignant tumor in the body.
• Diseases of the connective tissue, including: systemic lupus erythematosus, rheumatic fever, systemic scleroderma, etc.
• Previous infections, such as mycoplasmosis and cytomegalovirus infection.
• Autoimmune diseases not associated with damage to the hematopoietic system, for example, thyroiditis, Crohn's disease, sarcoidosis, etc.
• Type 1 diabetes mellitus.
• Treatment with antibiotics from the cephalosporin or penicillin group.
• Immunodeficiency states.

More often than others, the thermal form of autoimmune anemia occurs, when the internal environment of the body has normal temperature values, and class G immunoglobulins, as well as components C3 and C4, are located on erythrocytes. The destruction of erythrocytes occurs only in the spleen with the participation of macrophages.

The cold form of autoimmune anemia can have an unknown cause, or develop against the background of infection (with mononucleosis or mycoplasma pneumonia), against the background of hypothermia and with lymphoproliferative diseases. In the latter case, people over 60 suffer. A pathological reaction in the body, accompanied by the destruction of red blood cells, manifests itself after the temperature in the peripheral vessels drops to 32 ° C or less. Class M immunoglobulins are cold autoagglutinins.

The hemolysis that occurs in the spleen is often very severe. And sometimes it is not possible to save the patient.

The course of anemia provoked by infections is most often acute. If the violation was caused by an unknown cause, then it becomes chronic.

A rare form of autoimmune anemia is paroxysmal cold anemia. In this case, hemolysis develops when the body is exposed to cold. Even taking cold drinks and washing your hands in cool water is dangerous. Sometimes such anemia is diagnosed against the background of syphilis. The severity of the course of the disease varies from case to case. Sometimes an incurable form of pathology can be observed, leading to a fatal outcome.

Symptoms of autoimmune anemia

Autoimmune anemia is characterized by two syndromes: anemic and hemolytic.

The following signs indicate the development of anemic syndrome:

• Pale skin and mucous membranes.
• Dizziness attacks.
• Frequent nausea.
• Increased heart rate.
• Weakness that occurs regardless of the degree of physical or mental stress on a person.
• Increased fatigue.

Hemolytic syndrome is expressed by the following symptoms:

• Depending on the intensity of hemolysis, the skin may become light yellow or dark yellow.
• The spleen increases in size, painful sensations begin to disturb in the left hypochondrium.
• The urine turns brown.
• DIC may develop with the formation of multiple blood clots and bleeding that is difficult to stop.

Acute anemia most often develops against the background of an infectious infection of the body. Therefore, in addition to signs of destruction of erythrocytes, a person will develop symptoms of the underlying disease.

Cold autoimmune anemia is characterized by a chronic course. When exposed to low temperatures on the human body, he has a blanching of the fingers and toes, ears, face, as well as thrombophlebitis. Ulcers and even gangrene can form. Sometimes patients note the development of cold urticaria. Skin lesions persist for a long period of time.

Heat anemia also has a chronic course. An exacerbation of the pathological process occurs against the background of an increase in body temperature, which is often observed in viral and bacterial infections. A characteristic feature is urine staining black.

Acute autoimmune anemia is characterized by fever, chills, headaches, and dizziness. In parallel, shortness of breath joins, there are pains in the abdomen and in the lumbar region. The skin turns pale, may turn yellow, subcutaneous hemorrhages appear on the limbs. In addition to the spleen, the liver increases in size.

In the chronic course of the pathological process, a person feels satisfactory. An increase in the size of the spleen and recurrent jaundice may indicate an existing disorder. Attacks of remission alternate with bouts of exacerbation.

Diagnosis of autoimmune anemia

To make the correct diagnosis, an external examination of the patient is not enough. In addition to collecting anamnesis, blood donation will be required. A blood test indicates an increase in ESR indicators, reticulocytosis, normo- or hypochromic anemia, and an increase in the level of bilirubin in the blood are also detected. At the same time, the level of hemoglobin and erythrocytes decreases.

It is imperative to pass urine for analysis. It will detect protein, excess hemoglobin and urobilin.

Also, the patient is sent for an ultrasound scan of the internal organs with a study of the state of the liver and spleen.

If the data obtained is not enough to make the correct diagnosis, then the bone marrow must be taken, for which its puncture is performed. After examining the material obtained, it will be possible to detect hyperplasia of brain tissue, which occurs due to the activation of erythropoiesis. A diagnostic procedure called trepanobiopsy has a similar purpose as a bone marrow puncture. However, it is more difficult to tolerate by patients, therefore it is rarely used.

A direct Coombs test for autoimmune anemia will be positive. However, when negative test results are obtained, autoimmune anemia cannot be ruled out. This is often observed during treatment with hormonal drugs or with too intense hemolysis.

An enzyme-linked immunosorbent assay allows you to identify the class and type of immunoglobulins that are involved in an autoimmune reaction.

Autoimmune anemia treatment

Treatment of anemia of an autoimmune nature is most often long-term and does not always end with a complete recovery of the patient. First you need to decide on the reasons that led to the fact that the body began to destroy its own erythrocytes. If the etiological factor can be identified, then efforts should be made to eliminate it.

If the cause remains unknown, that is, the diagnosis sounds like "idiopathic autoimmune anemia", then the patient is prescribed drugs from the group of glucocorticosteroids. The drug of choice is Prednisolone. If the course of anemia is severe and the level of hemoglobin in the blood decreases to 50 g / l, then it is impossible to do without transfusion of erythrocyte mass.

Blood detoxification is performed in order to remove the breakdown products of red blood cells from it and improve a person's well-being. Plasmapheresis makes it possible to reduce the level of antibodies that circulate in the bloodstream. Symptomatic treatment is mandatory. To prevent the development of DIC syndrome, the patient is prescribed indirect anticoagulants. To support the hematopoietic system, the introduction of vitamin B12 and folic acid is indicated.

If you managed to cope with the disease, then this is where the therapy ends. When autoimmune anemia reappears after some time, the patient is referred for surgery to remove the spleen. This will prevent the development of hemolytic crises in the future, since it is the spleen that is the "graveyard" for erythrocytes in the human body. This procedure leads to a complete recovery quite often, in about 74-85% of cases.

Immunosuppressive therapy is an extreme measure in the treatment of autoimmune anemia, which is used only when splenectomy does not achieve the desired results.

Prevention and prognosis of the disease

In order to prevent the development of anemia, it is necessary to direct efforts to prevent human infection with dangerous viruses that can provoke illness. If anemia has already developed, then you should minimize the impact on the body of those factors that can cause its aggravation, for example, avoiding high or low temperatures.

It is impossible to prevent the development of idiopathic anemia, as its causes are unknown.

If the patient has suffered an episode of autoimmune anemia at least once, then the next two years he must donate blood for a general analysis. This should be done at intervals of 3 months. Any symptoms that may indicate re-developing anemia should be evaluated immediately by a physician.

In terms of prognosis, idiopathic anemia is more difficult to treat. No more than 10% of patients can achieve complete recovery after undergoing a hormonal course. However, removing the spleen increases the number of people who have recovered by up to 80%. It is difficult for patients to tolerate immunosuppressive therapy; such treatment negatively affects immunity and causes multiple complications. The success of treatment largely depends on the factor that caused the development of anemia.

The author of the article: Shutov Maxim Evgenievich | Hematologist

Education: In 2013 he graduated from the Kursk State Medical University and received a diploma "General Medicine". After 2 years, completed residency in the specialty "Oncology". In 2016 completed postgraduate studies at the National Medical and Surgical Center named after N. I. Pirogov.