Stroke in a Child with Hemoglobin SC Disease: A Case Report Describing use of Hydroxyurea after Transfusion Therapy

Area of Science:

• Pediatric Neurology
• Hematology
• Vascular Medicine

Background:

• Hemoglobin SC (HbSC) disease is a hemoglobinopathy associated with silent cerebral infarcts but a low incidence of overt stroke.
• Cerebrovascular complications in HbSC disease require careful monitoring and management.

Observation:

• A 2-year-old African American boy with a history of HbSC disease presented with acute focal neurologic deficits.
• Magnetic resonance imaging revealed cerebral infarction and associated vascular abnormalities.

Findings:

• This case represents a rare occurrence of stroke in a pediatric patient with HbSC disease.
• The patient was initially treated with monthly transfusions and later transitioned to hydroxyurea therapy.
• Hydroxyurea's efficacy as a fetal hemoglobin inducer in ameliorating clinical symptoms in HbSC disease is supported by retrospective data.

Implications:

• This case underscores the importance of recognizing stroke as a potential complication in pediatric HbSC disease, despite its rarity.
• The successful use of hydroxyurea therapy suggests its potential role in managing cerebrovascular events in this population.
• Further research into the long-term benefits of hydroxyurea in preventing stroke in HbSC disease is warranted.