What would the electrophoresis results of a patient with Beta-Thalassemia major reveal?

In a patient with Beta-Thalassemia major, the key electrophoresis finding is the presence of elevated levels of hemoglobin F (fetal hemoglobin) and a significant reduction or absence of hemoglobin A (which is composed of two alpha and two beta globin chains). While "little or no hemoglobin alpha" may seem plausible because of the absence of sufficient beta chains, it’s the absence of hemoglobin A that is most characteristic of the condition.

In Beta-Thalassemia major, the mutation leads to impaired production of beta globin chains; hence, individuals primarily produce hemoglobin F, which consists of two alpha and two gamma globin chains. This compensatory increase in hemoglobin F occurs while hemoglobin A is low or absent.

Overall, the hallmark of this disorder on electrophoresis would reflect a pattern where hemoglobin F is significantly elevated, demonstrating the body's response to the lack of beta chain production. This abnormal hemoglobin synthesis is integral to understanding Beta-Thalassemia and explains the correct interpretation of electrophoresis findings in this condition.