EHA issues HbSC-specific management guidelines for children and adults

The European Hematology Association (EHA) has developed clinical practice guidelines specifically for children and adults with hemoglobin SC (HbSC) disease, published in HemaSphere. HbSC is the second most common form of sickle cell disease in populations of African origin, but its distinct clinical and laboratory features have not previously been addressed in dedicated management guidelines, according to the abstract.

Developed under the EHA Guidelines Methodology Framework, the guidance involved hematologists, pediatricians, methodologists and patient representatives. Thematic working groups reviewed relevant English-language studies identified through PubMed and drafted recommendations. A modified Delphi process used anonymous online voting, with adoption requiring at least 70% agreement and less than 15% disagreement. The panel adopted 35 recommendations, including guidance on hydroxyurea and therapeutic venesection.

Compared with HbSS disease, HbSC generally involves higher hemoglobin levels, fetal hemoglobin below 5%, and fewer vaso-occlusive and vasculopathic complications, although retinopathy is more common. These differences support disease-specific management guidance. However, the available abstract does not provide individual recommendations, treatment eligibility criteria or evidence-strength assessments; the full guideline is needed to interpret and apply its advice clinically.