Which hemoglobin electrophoresis findings suggest sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

In children with normocytic hemolytic anemia, if results of electrophoresis show only HbS with an HbF concentration of less than 30%, the diagnosis is sickle cell anemia. If HbS and HbC are present in roughly equal amounts, the diagnosis is HbSC disease.

In children with microcytic hemolytic anemia, order quantitative Hb A2 in addition to electrophoresis. If HbS is predominant, Hb F is less than 30% and Hb A2 is elevated, a diagnosis of HbS–beta-0 thalassemia can be inferred. If possible, perform a study of the parents. If the HbA2 level is normal, consider the possibility of concomitant HbSS and iron deficiency. If HbS is greater than A and HbA2 is elevated, a diagnosis of HbS–beta+ thalassemia can be inferred. If HbS and HbC are present in equal amounts, the diagnosis is HbSC disease.

A homozygous patient will have hemoglobin SS (HbSS, 80-90%), hemoglobin F (HbF, 2-20%), and hemoglobin A2 (HbA2, 2-4%). A carrier patient will have HbSS (35-40%) and hemoglobin A (HbA, 60-65%). The test is not accurate in a patient who has recently received blood transfusions.


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