Description
The type of Hemoglobin that an individual has is determined by the Hemoglobin gene that they have inherited from both their parents. The normal adult Hemoglobin which predominates in individuals over the age of one year is called Hemoglobin ‘A’ (HbA). It is made up of a pair of α and a pair of β chains. Synthesis of the β chain is controlled by the β globin gene located on chromosome 11, whilst production of β chains is controlled by the β globin gene located on chromosome 16. The β gene is expressed once on each of chromosome 11 but the β gene is expressed twice on each of chromosome 16 and therefore synthesises a double amount of β chains. Hemoglobinopathies refer to a range of genetically inherited disorders of Hemoglobin and includes sickle cell disease and the thalassemias. Sickle cell disease and β thalassemia major are two of the commonest forms of these genetically inherited disorders.
Indications
This is a semiquantitative test used for the diagnosis of Hemoglobinopathies including thalassemias, sickle cell anemia, the detection of Hemoglobin C, D, E, F and other rare types of Hemoglobin.
Sample Type, Quantity & Conditions
2 ml EDTA Whole Blood Stability: 4 Days at 2-8 °C
Special Precautions
HPLC and Capillary methods electrophoresis are performed. Blood transfusions may give false or inconsistent results.
Normal Range
HbA: 94.0 - 98.5 % Hb A2 1.5 - 3.5 % Hb F: up to 2.0 % Hb S: Negative Hb C: Negative Hb D: Negative
