New: NCI-funded clinical trial search
Mednet Logo
CommunityPediatric Hematology/Oncology

How would you approach secondary stroke prevention in an adult with Hemoglobin SC disease?

Would you consider chronic RBC exchange versus HU?
1 Answer
Mednet MemberInvited Expert
Hematology · Boston University School of Medicine
Answered on

Stroke is less common in HbSC disease than it is in HbS homozygotes (Ohene-Frempong et al., PMID 9414296). Thus, there are no studies focused on primary or secondary stroke prevention in HbSC disease. Recent guidelines for stroke management were “silent” on stroke in HbSC disease (DeBaun et al., PMID 32298430). Hydroxyurea almost always fails to induce high levels of HbF in HbSC disease, especially in adults. I would not recommend its use for secondary prevention. Assuming the stroke was ischemic, the best course specific for sickle cell disease would be transfusion, probably exchange transfusion, to reduce the concentrations of HbS and HbC to about 30% of total hemoglobin at the time of the next transfusion. These recommendations are conti...

Join for free or sign in to see the full answer