High risk during surgery:

 # Which of the following may constitute high-risk during surgery? 

a. β-Thalasemia minor 

b. Hb S homozygous 

c. Hb D Punjab

d. Hb E trait

 

The correct answer is B. Hb S homozygous.

Hb D Punjab and Hb D Los Angeles are having same biochemical structure (Glutamic acid is replaced by Lysine at 121 position in β chain). Heterozygous state is essentially asymptomatic. Homozygous Hb D is very rare. Sickle cell disease is relatively less associated with by Hb S/D Punjab/Los Angeles . Mutation in β chain by virtue of which glutamic acid is replaced by lysine at 26th position in β chain causes Hb E. In Hb E carrier state, 30–45% of the Hb is Hb E, and such carriers are asymptomatic but shows microcytosis. Homozygons E disease is associated with marked microcytosis and hypochromia, but the anemia is usually mild. Clinically resembles β-thalassemia minor. Hb E with Hb D heterozygous state is variable in severity.

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