Which common clinical feature is associated with sickle cell disease?

Sickle cell disease is characterized by the production of abnormal hemoglobin, known as hemoglobin S. This defective hemoglobin causes red blood cells to deform into a sickle shape, particularly under low oxygen conditions. These sickled cells have a shorter lifespan than normal red blood cells, leading to a state of chronic hemolytic anemia. Anemia is a hallmark of sickle cell disease because the body does not produce enough healthy red blood cells to replace the ones that are destroyed.

This chronic anemia can manifest with symptoms such as fatigue, pallor, and shortness of breath, among others. Additionally, frequent pain crises can occur due to vaso-occlusive events caused by the sickled cells obstructing blood flow in small vessels, which further exacerbates anemia. Although other clinical features might be associated with the disease, such as pain crises, infections, and splenic dysfunction, anemia stands out as a fundamental and common feature that directly stems from the pathophysiology of the disorder.