Knowledge insufficient: the management of haemoglobin SC disease

2016/12/16 by Lydia H. Pecker, Beverly A. Schaefer, Lori Luchtman‐Jones · 66 citations
Medicine · #Blood groups and transfusion #Disease #Disease management #Hemoglobinopathies and Related Disorders #Intensive care medicine #Internal medicine #Iron Metabolism and Disorders #Medicine

paper · pdf · doi:10.1111/bjh.14444

published in British Journal of Haematology 176(4), 515-526 (Wiley)

openalex publication_date 2016/12/16 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/27

Abstract

Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. These studies are underpowered to allow definitive conclusions about HbSC. We review the pathophysiology of HbSC disease, including known and potential differences between HbSS and HbSC, and highlight knowledge gaps in HbSC disease management. Clinical and translational research is needed to develop targeted treatments and to validate management recommendations for efficacy, safety and impact on quality of life for people with HbSC.

Citations

• The effects of hydroxycarbamide and magnesium on haemoglobin SC disease: results of the multi‐centre CHAMPS trial

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