EHA Guidelines on the management of children and adults with hemoglobin SC disease

HbSC disease is the second most common type of sickle cell disease (SCD) in populations of African origin. Although it is often grouped together with other types of SCD, particularly sickle cell anemia (HbSS), it has unique pathophysiology and a different spectrum of laboratory and clinical features. Compared to HbSS, hemoglobin levels are higher, HbF is lower at less than 5%, and most vaso-occlusive and vasculopathic complications are less frequent, although retinopathy is more common. Despite these differences, there have been no specific guidelines on the management of HbSC disease. This clinical practice guideline was developed by the European Hematology Association (EHA) in accordance with the EHA Guidelines Methodology Framework to provide consensus-based guidance for the management of children and adults with HbSC disease. A multidisciplinary Guideline Panel was convened by the EHA Guidelines Committee and included hematologists, pediatricians, methodologists, and patient representatives with expertise in SCD and its complications. Panel members were assigned to thematic working groups based on their clinical expertise, and each group was responsible for drafting content and recommendations within their respective domains. Literature searches were conducted using PubMed for relevant studies published in English. A modified Delphi process was used to reach consensus on recommendations, and panel members voted anonymously using an online system. A predefined threshold of ≥70% agreement and <15% disagreement was required for adoption. Statements not reaching consensus were revised and subjected to further rounds of discussion and voting. Thirty-five recommendations were adopted, including guidance on the use of hydroxyurea and therapeutic venesection.