What is the role of a sickle cell trait in the pathophysiology of beta thalassemia?

Updated: Dec 19, 2018
  • Author: Pooja Advani, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

The coexistence of sickle cell trait and beta thalassemia is a major and symptomatic hemoglobinopathy with most of the symptoms and complications of sickle cell disease. Unlike sickle cell trait, in which most Hb-on-Hb electrophoresis is Hb A (AS), S is the dominant Hb (SA) and usually constitutes about 60% or more of the circulating Hb, depending on the transfusion status of the patient and the nature of the coexisting beta-thalassemia mutation (ie, beta-zero vs beta-plus).


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