Anemias caused by the carriage of abnormal unstable hemoglobins
Jun 18, 2018
Unstable hemoglobins are understood as such anomalous hemoglobins, which due to the instability of the molecules precipitate in the erythrocyte, which leads to anemia.
In 1952, a sick child with congenital hemolytic anemia was first described in the literature, after which the Heinz body was found in all erythrocytes after removal of the spleen. Only 18 years later, this child had the cause of the formation of Heinz bodies - an abnormal fraction of hemoglobin. In the beginning, this group of diseases was called "congenital hemolytic anemia with Heinz bodies".The first unstable hemoglobin with the established structure was discovered in 1962 in 2 persons from the Swiss family. They suffered an acute hemolytic crisis( massive destruction of erythrocytes with all the characteristic manifestations: deep anemia, jaundice, intoxication, rise in body temperature, etc.) after taking sulfanilamide preparations. In 1964 it was found out that a part of hemoglobin with Heinz bodies easily precipitates with little heating. In 1969, a new name was proposed for the entire group of anemia - "hemolytic anemia caused by the carriage of unstable hemoglobins."
The disease is transmitted by a dominant type of inheritance. Heinz's calves are attached to the inner surface of the erythrocyte membrane. This changes the shape and flexibility of red blood cells. The spleen dislocates the body of Heinz from the erythrocyte, the erythrocytes lose part of their surface, and the duration of their life is shortened. After removal of the spleen in the erythrocytes, the bodies of Heinz appear, which before the operation are often not determined, while the life span of red blood cells is prolonged.
Manifestations of the disease are very diverse. In some, the hemoglobin content may remain normal, others have severe anemia with a decrease in hemoglobin content of up to 40-60 g / l. Severe or moderate-to-severe hemolytic anemia has been detected since childhood. The severity of jaundice and anemia varies. In some individuals, skin color and sclera are normal, others have permanent or periodic yellowing. The spleen is in most cases increased, but with some forms of the disease may remain normal. A number of people have a liver. Anemia is often complicated by cholelithiasis. Changes in the skeleton can be the same as in microspherocytosis, and for mild forms of the disease they are absent.
Anemia of different severity is detected in the blood. The content of immature forms of red blood cells is always increased.
The detection of the disease is based on the detection of abnormal unstable hemoglobins by special methods.
Treatment of .Some people with unstable hemoglobins do not need any treatment. Persons with significant manifestations of the disease undergo surgery to remove the spleen. Useful riboflavin( vitamin B2) or its active drugs.