A 25-year-old woman with sickle cell disease (HbSS) has 4 vaso-occlusive crises per year and is not on disease-modifying therapy. Which agent, approved by FDA for adults with SCA to reduce vaso-occlusive crises, works by inhibiting P-selectin?

• A Hydroxyurea
• B Voxelotor
• C L-glutamine
• D Crizanlizumab ✓

Explanation

Crizanlizumab is a monoclonal antibody against P-selectin, a cell adhesion molecule expressed on activated platelets and endothelial cells that mediates red cell-white cell-endothelium interactions in sickle cell vaso-occlusion. The SUSTAIN trial demonstrated a significant reduction in the median annual rate of vaso-occlusive crises (from 2.98 to 1.63 per year, ~45% reduction). FDA approved it for adults and pediatric patients ≥16 years with SCA. Voxelotor inhibits HbS polymerisation; hydroxyurea increases HbF; L-glutamine reduces oxidative stress.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.