Anemia caused by abnormal carriage unstable hemoglobins
Under unstable hemoglobins understand such abnormal hemoglobins, which, due to the instability of the molecules precipitate in the red blood cell, which leads to anemia.
In 1952, in the literature was first described by a sick child with congenital hemolytic anemia, which after removal of the spleen were found in all red blood cells Heinz calf.Only after 18 years, that child has been installed Taurus Education reason Heinz - abnormal hemoglobin fraction.Initially, this group of diseases called "congenital hemolytic anemia with Heinz calves."First unstable hemoglobin installed structure was discovered in 1962 from 2 persons from a Swiss family.They suffered an acute hemolytic crisis (massive destruction of red blood cells with all the characteristic symptoms: profound anemia, jaundice, intoxication, the rise in body temperature, etc...) After taking sulfa drugs.In 1964, it was found that the hemoglobin part corpuscles Heinz easily precipitates under slight heating.In 1969, it was proposed a new name for
disease is transmitted by dominant inheritance type.Tau Heinz attached to the inner surface of the erythrocyte membrane.This changes the shape and flexibility of red blood cells.Spleen knocks of erythrocyte calf Heinz, the red blood cells lose part of its surface, and their life expectancy is shortened.After removal of erythrocytes in the spleen appear Heinz cells that often do not pre-operatively determined, thus lengthening the duration of life of erythrocytes.
very diverse manifestations of the disease.Some hemoglobin may be normal, others have severe anemia with decreased hemoglobin to 40-60 g / l.Heavy to moderate hemolytic anemia detected since childhood.Severity of jaundice and anemia varies.In some individuals, the skin color and normal sclera, others noted the constant or intermittent severe yellowness.Spleen increased in most cases, but in some forms of the disease may remain normal.A number of individuals enlarged liver.Anemia is often complicated by cholelithiasis.Changes in the skeleton may be the same as in microspherocytosis, while they are absent milder forms of the disease.
In the blood revealed anemia of varying severity.The content of the immature forms of erythrocytes always increased.
Identification of disease based on the detection of special methods of investigation of abnormal unstable hemoglobins.
Treatment .Some individuals with unstable hemoglobins do not need any treatment.Individuals with significant manifestations of the disease is carried out an operation to remove the spleen.Useful riboflavin (vitamin B2) or active agents.