Practice question
Question
Which of the following statements are correct for sickle cell disease? (A) Hbs differs from normal HbA by a single amino acid substitution (B) The sickling effect of red blood corpuscles is the result of abnormal Hb binding with the red cell membrane (C) Splenic infarction may occur but this has little clinical consequence (D) Life-threatening thromboses can occur Choose the correct answer from the options given below:
Explanation
HbS results from β6 Glu→Val substitution causing deoxy-HbS polymerization, not membrane binding. Polymerized fibers distort RBCs into sickles, precipitating vaso-occlusive pain, splenic infarctions causing autosplenectomy with severe infection risk, and thrombotic crises, so A and D are true.
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