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Hemoglobin electrophoresis is a blood test that can detect different types of hemoglobin. The test can detect hemoglobin S, the form associated with sickle cell disease, as well as other abnormal types of hemoglobin, such as hemoglobin C. It can also be used to investigate thalassemias, which are disorders caused by defective hemoglobin production.
A schematic of hemoglobin electrophoresis, showing the banding which is typical of various types of hemoglobin. Note that sickle cell disease (SCD) gives a single, bold band whereas sickle cell trait gives two slightly fainter bands. Where SCD is suspected, a number of tests can be used.
The deoxygenation of hemoglobin in sickle cell patients has severe implications on those who carry the mutation. The hemoglobin proteins, present in those with sickle cell disease, cannot carry oxygen to the organs and other tissues of the human body. This results in pain crises and the disease results in an abbreviated life expectancy of 40 ...
Some findings also show the reduction of the sickle-cell trait in those who retain much more fetal hemoglobin than usual in adulthood. Fetal hemoglobin likely plays a role in the prevention of sickling. Elevated fetal hemoglobin levels have been observed in populations where sickle-cell disease is prevalent. [20] [5] [21]
The hemoglobin structural variants can be broadly classified as follows: [19] Sickle cell disorders, which are the most prevalent form of hemoglobinopathy. Sickle hemoglobin (HbS) is prone to polymerize when deoxygenated, precipitating within the red blood cell. This damages the RBC membrane resulting in its premature destruction and consequent ...
Adult hemoglobin (HbA) is produced at low levels through embryonic and fetal life and is the predominant hemoglobin in children by six months of age and onward; it constitutes 96-97% of total hemoglobin in individuals without a hemoglobinopathy. It is composed of two alpha globins and two beta globins (α2β2).