Cert Clin Haematology(SA) Paed Practice Questions - CMSA Sub-specialty Certificate In Clinical Haematology (Paediatrics) Exam

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A 4-year-old child with sickle cell anaemia (HbSS) is initiated on hydroxyurea disease-modifying therapy. Which physiological mechanism accounts for the primary therapeutic benefit of hydroxyurea in reducing vaso-occlusive pain crises?

Induction of gamma-globin gene expression and increased fetal hemoglobin (HbF) Competitive inhibition of 2,3-bisphosphoglycerate (2,3-BPG) synthesis Direct inhibition of sickle hemoglobin polymer cross-linking Irreversible binding to RBC membrane Band 3 protein to enhance deformability

Correct Answer:

Induction of gamma-globin gene expression and increased fetal hemoglobin (HbF)

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