A 5-year-old boy with HbSS sickle cell disease is screened annually. Transcranial Doppler shows a time averaged mean maximal velocity of 215 cm/s in the right middle cerebral artery. He has never had a stroke and is clinically well. What is the appropriate intervention?

• A Start hydroxyurea and repeat Doppler in six months
• B Initiate a program of regular blood transfusions to keep HbS below 30% ✓
• C Refer immediately for hematopoietic stem cell transplantation
• D Begin daily aspirin and continue annual Doppler surveillance

Explanation

A time averaged mean maximal velocity above 200 cm/s in the distal internal carotid or middle cerebral artery identifies children with HbSS at very high risk of overt stroke within a few years. Standard care is a chronic transfusion program targeting suppression of HbS to under 30%, shown to cut stroke risk dramatically. Hydroxyurea reduces painful crises but does not replace transfusion once the high risk threshold is crossed. Transplantation is reserved for selected cases and aspirin has no proven role in this setting.

Reference: Nelson Textbook of Pediatrics, 22nd ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.