A 26-year-old man undergoing pre-employment screening has Hb 14.2 g/dL, normal MCV, and a normal blood film apart from rare ovalocytes. Hemoglobin electrophoresis shows HbA 58%, HbS 39%, HbA2 2%, HbF 1%. Appropriate counseling should include:

• A Starting hydroxyurea to raise HbF levels
• B Avoiding intense exertion with dehydration and extremes of altitude or temperature ✓
• C Annual exchange transfusion to prevent vaso-occlusive events
• D Pneumococcal vaccination and lifelong penicillin prophylaxis

Explanation

The pattern of roughly 60% HbA and 40% HbS with normal hemoglobin defines sickle cell trait. Most carriers are asymptomatic, but extreme conditions such as exhaustive exercise with dehydration, high altitude, or severe hypoxia can trigger sickling, renal medullary carcinoma risk, and rare exertional deaths. Hydroxyurea, transfusion programs, and penicillin prophylaxis apply to homozygous sickle cell disease, not to the trait.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.