How does alpha thalassemia affect hemoglobin?
In people with the characteristic features of alpha thalassemia, a reduction in the amount of hemoglobin prevents enough oxygen from reaching the body’s tissues. Affected individuals also have a shortage of red blood cells (anemia ), which can cause pale skin, weakness, fatigue, and more serious complications.
What does hemoglobin S stand for?
Hemoglobin S (Hgb S) is an abnormal type of hemoglobin that you can inherit from your parents. Hgb S causes red blood cells to become stiff and abnormally shaped. Instead of having a normal round, disk shape, these red blood cells become sickle-shaped, or crescent-shaped.
Which hemoglobin is increased with thalassemia?
The distinguishing finding in beta thalassemia is a hemoglobin electrophoresis with the finding of elevated Hgb A2 and F. Both will be increased in beta thalassemia trait without iron deficiency, and will be normal or decreased in alpha thalassemia and isolated iron deficiency anemia.
What is the difference between thalassemia and alpha thalassemia?
The thalassemias are a group of inherited hematologic disorders caused by defects in the synthesis of one or more of the hemoglobin chains. Alpha thalassemia is caused by reduced or absent synthesis of alpha globin chains, and beta thalassemia is caused by reduced or absent synthesis of beta globin chains.
Which hemoglobin S can be decreased in alpha thalassemia?
α-Thalassemia results in both a decreased erythrocyte total hemoglobin content and an increased amount of hemoglobin F, which serves not only to decrease the relative percentage of hemoglobin S within the RBCs, but also to maintain a higher oxygen tension, since F is a high oxygen affinity hemoglobin.
How is hemoglobin S detected?
Sickle cell tests determine the presence and relative amount of hemoglobin S in a blood sample or detect mutations in the genes that produce hemoglobin to help diagnose sickle cell anemia and/or identify people with sickle cell trait. Hemoglobin is the protein in red blood cells (…
Is Hemoglobin E trait a thalassemia?
Hemoglobin E (HbE) is an extremely common structural hemoglobin variant that occurs at high frequencies throughout many Asian countries. It is a β-hemoglobin variant, which is produced at a slightly reduced rate and hence has the phenotype of a mild form of β thalassemia.
What does low Hemoglobin A2 mean?
Low Hb A2 percentages were found in iron-deficiency anemia, hereditary persistance of fetal hemoglobin, and Hb H disease. In iron-deficiency anemia, the lowest levels of Hb A2 were observed in association with the most severe anemia.
What blood tests are done to detect thalassemia?
Abnormally shaped red blood cells are a sign of thalassemia. The lab technician may also perform a test known as hemoglobin electrophoresis . This test separates out the different molecules in the red blood cells, allowing them to identify the abnormal type.
What is hemoglobin’s?
Medical Definition of hemoglobin S. : an abnormal hemoglobin occurring in the red blood cells in sickle cell anemia and sickle-cell trait and differing from hemoglobin A in having a valine residue substituted for the glutamic-acid residue in position 6 of two of the four polypeptide chains making up the hemoglobin molecule.
What is alpha thalassemia vs. Beta thalassemia?
There are two major forms of thalassemia as alpha thalassemia and beta thalassemia. In alpha thalassemia, there is a decrease in the number of alpha globin chains whereas in beta-thalassemia it is the number of beta globin chains that goes down. This is the key difference between alpha and beta thalassemia.
Is alpha thalassemia life threatening?
Alpha-Thalassemia is one of the two forms of the life threatening blood disease known as Thalassemia. Read on to know all about its various types, symptoms and possible treatment options.