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Types of sickle cell disease

Sickle cell disease (SCD) is a group of inherited red blood cell disorders, and there are several different types of sickle cell disease based on the specific genetic mutations involved. Here are the main types:

1. Hemoglobin SS Disease (HbSS)

This is the most common and severe form of sickle cell disease. It occurs when a person inherits two sickle cell genes (one from each parent), leading to the production of only sickle hemoglobin (HbS). This form is commonly referred to as sickle cell anemia and is associated with more severe symptoms, such as frequent pain crises, anemia, and a higher risk of complications.

2. Hemoglobin SC Disease (HbSC)

This form occurs when a person inherits one sickle cell gene and one gene for hemoglobin C (a different abnormal hemoglobin). While the symptoms of HbSC are generally milder than those of HbSS, individuals with this form can still experience pain crises, anemia, and other complications.

3. Hemoglobin S/β-Thalassemia (HbS/β-Thal)

This type of sickle cell disease results from the inheritance of one sickle cell gene and one beta-thalassemia gene, which affects hemoglobin production. The severity of symptoms depends on whether the beta-thalassemia gene reduces or completely stops hemoglobin production.

  • HbS/β0-thalassemia: This is a more severe form, as the body produces almost no normal hemoglobin.

  • HbS/β+-thalassemia: This is milder, as the body produces some normal hemoglobin in addition to the sickle hemoglobin.

4. Hemoglobin SD Disease (HbSD)

This form occurs when a person inherits one sickle cell gene and one gene for hemoglobin D. Hemoglobin D is a less common variant, and individuals with HbSD typically have milder symptoms compared to those with HbSS, but can still experience complications like anemia and pain.

5. Hemoglobin SE Disease (HbSE)

While not a type of sickle cell disease, sickle cell trait occurs when a person inherits one sickle cell gene and one normal hemoglobin gene. People with sickle cell trait typically do not have symptoms of sickle cell disease but can pass the sickle cell gene to their children.

6. Sickle Cell Trait (AS)

While not a type of sickle cell disease, sickle cell trait occurs when a person inherits one sickle cell gene and one normal hemoglobin gene. People with sickle cell trait typically do not have symptoms of sickle cell disease but can pass the sickle cell gene to their children.

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