Autoimmune hemolytic anemia with incomplete heat agglutinins

What is autoimmune hemolytic anemia with incomplete heat agglutinins?

The clinical picture of autoimmune hemolytic anemia is identical in both idiopathic and symptomatic forms. The onset of the disease can be different, sometimes even very acute.

On the background of complete health, severe weakness develops, there may be back pain, pain in the region of the heart, shortness of breath, palpitations, and often a fever. Jaundice develops fairly quickly, which often directs the doctor to the wrong diagnosis of infectious hepatitis.

If the disease is of a gradual nature, then the immediate onset is preceded by symptoms such as joint pain, abdominal pain, fever.

Often the disease develops suddenly. Patients feel satisfactory.

Among the main signs of the disease are common symptoms of anemia: an increase in heart, systolic murmur at the apex and in the fifth point, tachycardia, dizziness, pallor, shortness of breath, and signs characteristic of hemolytic anemia: jaundice, an increase in liver size and spleen.

Diagnosis of autoimmune hemolytic anemia with incomplete thermal agglutinins

In cases of acute hemolytic crises, the level of hemoglobin in the blood is reduced to extremely low numbers (below 45 g / l). As a rule, in patients with this fall is not observed, and the hemoglobin content is not reduced so much (to 60-70 g / l). A number of patients with chronic autoimmune hemolytic anemia have a slight decrease in hemoglobin (up to 90 g / l). Anemia often normochromic or moderately hyperchromic. The content of reticulocytes is increased in the majority of patients. In some of them, the content of reticulocytes rises to very large numbers (up to 87). In patients with symptomatic forms of the disease, the content of reticulocytes is lower than in the idiopathic form. Sometimes with severe exacerbation of the disease, the content of reticulocytes is reduced to 0.1-0.3%. Sometimes with autoimmune hemolytic anemia, macrocytosis is observed, but nevertheless microspherocytosis is found more often. The number of microspherocytes is the same as in hereditary microspherocytosis. This symptom is not specific for this disease. In severe disease, fragmented, destroyed red blood cells are found.

In the bone marrow, in most cases, the red sprout is enlarged, but sometimes the number of erythrocaryocytes decreases. Probably, such crises are associated with a very large number of antibodies, as a result of which not only the erythrocytes of peripheral blood are destroyed, but also the erythrocaryocytes. It is possible that such crises, as well as microspherocytosis and sickle cell anemia, are based on parvovirus infection. The level of blood leukocytes depends on the disease, which formed the basis of autoimmune destruction of red blood cells.

It is necessary to emphasize that in the idiopathic form of autoimmune hemolytic anemia there is a significant fluctuation in the number of leukocytes: in acute forms

diseases up to 50-70 × 109 / l with a shift to promyelocytes, in chronic forms it increases slightly or remains within the normal range. Sometimes a pronounced decrease in leukocytes develops.

The number of platelets in most patients is normal or slightly reduced. However, a combination of autoimmune hemolytic anemia with severe autoimmune thrombocytopenia or simultaneous autoimmune damage of all three shoots is possible. In some cases, autoimmune hemolytic anemia begins simultaneously with autoimmune thrombocytopenia, in others, thrombocytopenia joins after a few months or years. In some patients, thrombocytopenia is detected at the onset of the disease, and over time anemia develops, and thrombocytopenia may no longer be present.

Osmotic resistance in autoimmune hemolytic anemia with heat agglutinins is reduced in most cases. The content of bilirubin in autoimmune hemolytic anemia often increased. According to our data, the content of bilirubin is increased in 68% of patients. Most often, this increase is relatively small – from 25 to 45 µmol / l, although in some patients it reaches 60 µmol / l. In patients with the idiopathic form of autoimmune destruction of erythrocytes, an increase in bilirubin is observed more often than in the symptomatic form. The increase in bilirubin is mainly due to indirect, not associated with glucuronic acid. It should be noted that the normal bilirubin content does not exclude autoimmune hemolytic anemia. An increase in the amount of direct or glucuronic acid-related bilirubin should lead the doctor to an idea of ​​concomitant hepatitis or blockage of the bile ducts.

Urobilin content is sometimes increased in the urine, however this increase is non-permanent and does not indicate hemolytic anemia. In the feces of patients, as a rule, a significant amount of stercobilin is found.

Plasma hemoglobin content can be increased due to the slight intravascular destruction of red blood cells in these patients, but more often it is normal. Sometimes with autoimmune hemolytic anemia with incomplete thermal agglutinins, hemoglobinuria is observed. Patients often identify changes in the composition of protein fractions, an increase in ESR.

In some patients, the content of globulins increases, usually due to the immunoglobulin fraction. Sometimes with autoimmune destruction of red blood cells, the Wasserman reaction is positive (diagnosis of syphilis).

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