Hydroxyurea or chronic exchange transfusions in patients with sickle cell disease: role of transcranial Doppler ultrasound in stroke prophylaxis.
Hydroxyurea is increasingly used in patients with sickle cell disease and acts by several underlying mechanisms. Its usage has been reported to eliminate transfusion needs in children with beta-thalassemia major. It has also been used in sickle cell disease patients on stroke prophylaxis with exchange transfusions who develop transfusional iron overload and is now being considered as a possible alternative to chronic transfusions for secondary stroke prophylaxis. Our case demonstrates a pitfall when using hydroxyurea without monitoring intracranial cerebral vessels for vasculopathic changes; presence of which can predict stroke risk and alert the need for chronic blood transfusions as primary stroke prophylaxis. Transcranial Doppler is a crucial investigation that can reveal elevated cerebral arterial flow velocities.
Suliman
Heba
H
Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman.
Wali
Yasser
Y
Al Saadoon
Muna
M
Zechariah
Mathew
M
William
Ranjan R
RR
Gujjar
Arunodaya
A
Pathare
Anil
A
D000755:Anemia, Sickle Cell / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D000986:Antisickling Agents / Q000627:therapeutic use*; D001783:Blood Flow Velocity; D002536:Cerebral Arteries / Q000503:physiopathology; D002560:Cerebrovascular Circulation; D002675:Child, Preschool; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D020521:Stroke / Q000000981:diagnostic imaging* / Q000503:physiopathology; D017585:Ultrasonography, Doppler, Transcranial
D000986:Antisickling Agents; D006918:Hydroxyurea