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Rabu, 09 April 2014

Thalassemia Mediterranean Blood Disease

Thalassemia is a potentially life-threatening genetic disease. There are two different types--alpha thalassemia and beta thalassemia--that are further classified according to the severity of the condition. People with thalassemia may experience mild to sever anemia.

Definition

    Thalassemias are a group of hereditary blood disorders in which the oxygen-carrying component of blood, hemoglobin, has abnormalities which result in the destruction of red blood cells.

Types

    Hemoglobin is composed of two proteins, alpha globin and beta globin. Thalassemia occurs when one of the four genes on the alpha globin, or one or both genes on the beta globin, are missing or mutated. Thus thalassemia is classified as either alpha thalassemia or beta thalassemia.

Prevalence

    Alpha thalassemia is more prevalent in people from African descent and those from the Middle East, South Asia and China. Beta thalassemia is most prevalent in people of Mediterranean descent.

Symptoms

    There are three types of beta thalassemia. Symptoms range from mild anemia in thalassemia minor to moderately severe anemia and other health problems like an enlarged spleen and bone deformities in thalassemia intermedia. Thalassemia major results in life-threatening anemia which will require lifelong blood transfusions.

Treatment

    Depending on the severity, thalassemia patients undergo red blood cell transfusions every two to three weeks and are also given folic acid supplements. Patients will also require chelation therapy to remove the buildup of iron in the body.

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