A 30-year-old woman from West Africa presents with episodes of severe bony pain (hand-foot syndrome), recurrent fever, and jaundice since childhood. Hemoglobin is 7.5 g/dL, MCV 82 fL, reticulocyte count 12%, peripheral smear shows sickle-shaped cells and Howell-Jolly bodies. Which of the following treatments has been shown to reduce the frequency of painful crises in sickle cell disease?

• A Hydroxyurea ✓
• B Ferrous sulfate supplementation
• C Cobalamin (vitamin B12) injections
• D Deferoxamine infusion

Explanation

Hydroxyurea (hydroxycarbamide) increases fetal hemoglobin (HbF) synthesis, which inhibits HbS polymerization and reduces sickling. The MSH trial and subsequent studies demonstrated that hydroxyurea reduces the frequency of painful crises, acute chest syndrome, and hospitalizations, and improves survival in sickle cell disease. It is the first-line disease-modifying therapy. Ferrous sulfate and B12 are for nutritional anemias; deferoxamine is for iron overload (not indicated in non-transfused SCD without iron overload).

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.