In patients with sickle cell disease, what is a notable finding on hemoglobin electrophoresis?

In patients with sickle cell disease, hemoglobin electrophoresis reveals a notable finding of reduced hemoglobin A. This is primarily due to the genetic mutation that causes sickle cell disease, in which there is an abnormality in the beta-globin chain of hemoglobin, leading to the production of hemoglobin S (HbS) instead of the normal hemoglobin A (HbA).

Typically, patients with sickle cell disease inherit two copies of the sickle cell gene (one from each parent), resulting in a high proportion of HbS in their blood. Consequently, this significantly decreases the amount of normal hemoglobin A, which is what is detected during hemoglobin electrophoresis. Alongside HbS, patients may also have some percentage of fetal hemoglobin (HbF) since its production can increase in response to the disease. Therefore, the hallmark finding in sickle cell disease is the reduced presence of hemoglobin A and the predominance of hemoglobin S on electrophoresis.