Thursday, September 29, 2022

Alpha-thalassemia

Alpha thalassemia is a blood disorder that is inherited. Alpha thalassemia occurs when some or all of the 4 genes that make hemoglobin (the alpha-globin genes) are missing or damaged.

Alpha globin is a building block of hemoglobin. Hemoglobin is an iron-rich protein in red blood cells that carries oxygen to cells throughout the body.

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.

The alpha thalassemias can be generally categorized as: Silent Carrier, Alpha Thalassemia Trait, Hemoglobin H disease, Hemoglobin H-Constant Spring, and Alpha Thalassemia major.

A person who is a carrier has no signs or symptoms of anemia. They do not need any special medical treatment. However, they can pass the faulty genes on to their children. People who have moderate to severe forms of alpha thalassemia have inherited faulty hemoglobin genes from both parents.

Two types of alpha thalassemia can cause health problems. The more severe type is known as hemoglobin Bart hydrops fetalis syndrome, which is also called Hb Bart syndrome or alpha thalassemia major. The milder form is called HbH disease.
Alpha-thalassemia

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