Alpha-thalassemia
Alpha-thalassemia
Alpha-thalassemia (pronounced: al-fa thal-uh-SEE-mee-uh) is a blood disorder that reduces the production of hemoglobin. Hemoglobin is the protein in red blood cells that carries oxygen to cells throughout the body.
In people with alpha-thalassemia, a lack of alpha globin leads to a surplus of beta globin. The imbalance of globin chains can cause red blood cells to become misshapen and fragile, leading to anemia.
Etymology
The term "alpha-thalassemia" is derived from the Greek words "thalassa" meaning "sea" and "haima" meaning "blood". The "alpha" refers to the specific type of globin chain that is underproduced.
Types
There are four types of alpha-thalassemia, which are distinguished by their effects on hemoglobin:
- Alpha-thalassemia major is the most severe form of alpha-thalassemia and is usually fatal before or shortly after birth.
- Hemoglobin H disease is a moderate to severe form of alpha-thalassemia.
- Alpha-thalassemia intermedia is a moderate form of alpha-thalassemia.
- Alpha-thalassemia minor is a mild form of alpha-thalassemia.
Symptoms
The symptoms of alpha-thalassemia can vary depending on the specific type. They can range from no symptoms at all to severe life-threatening anemia. Other possible symptoms include fatigue, weakness, pale or yellowish skin, facial bone deformities, slow growth, abdominal swelling, and dark urine.
Treatment
Treatment for alpha-thalassemia also depends on the specific type. For milder forms, treatment may involve regular blood transfusions to provide the body with a normal supply of red blood cells. For more severe forms, treatment may involve a bone marrow transplant.
Related Terms
External links
- Medical encyclopedia article on Alpha-thalassemia
- Wikipedia's article - Alpha-thalassemia
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