How is HbS-beta 0 thalassemia diagnosed?

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

In HbS–beta 0 thalassemia, only HbS is found on electrophoresis. HbA2 is elevated and splenomegaly usually is present. The clinical picture is similar to SCD but is slightly less severe. Management is similar to that for SCD. In HbS–beta+ thalassemia, Hb A is present, usually between 10% and 30%. The spleen is usually enlarged. This disease is otherwise similar to SCD but is milder.


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