Table 1.
Types of Sickle Cell Disease
| Condition | Condition acronyms | Inheritance/Clinical manifestations |
|---|---|---|
| Homozygous sickle cell disease, sickle cell disease SS, or sickle cell anemia | HbSS or SCD-SS | Hemoglobin S gene is inherited from both parents. |
| Usually the most severe form of sickle cell disease. | ||
| Sickle hemoglobin C disease, hemoglobin HbSC disease or, sickle cell disease SC | HbSC or SCD-SC | Hemoglobin S gene is inherited from one parent and hemoglobin C gene is inherited from the other parent. |
| This is usually a milder form of the disease. | ||
| Sickle beta thalassemia disease: two major types |
Hemoglobin S gene is inherited from one parent and the beta thalassemia gene is inherited from the other parent. | |
| 1. Sickle beta zero thalassemia | HbS βo-thal or SCD-S βo-thal | People with HbS βo-thal usually have a more severe form; people with HbS β+- thal usually have a milder form. |
| 2. Sickle beta plus thalassemia | HbS β+-thal or SCD-β+-thal | |
| Rarer type examples: | In these rarer types a person inherits one sickle cell gene from one parent and one abnormal hemoglobin gene from the other parent. | |
| Hemoglobin SD disease or sickle cell disease SD | HbSD or SCD-SD | |
| Hemoglobin SE disease or sickle cell disease SE | HbSE or SCD-SE | The severity of these rarer types of the disease varies. For example, while HbSE parallels that of HbS β+-thal, HbSOArab and HbSCHarlem often have severe disease symptoms and complications similar to people with HbSS. HbS Lepore and HbSD have milder symptoms. |
| Hemoglobin SO Arab disease or Sickle cell disease SO Arab | HbSOArab or SCD-SOArab | |
| Hemoglobin SC Harlem or Sickle cell disease SC Harlem | HbSCHarlem or SCD-SCHarlem | |
| Hemoglobin sickle Lepore or Sickle cell disease Lepore | HbS Lepore or SCD-S Lepore |
Note: Adapted from: Pass et al. (2000).