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Knowledge insufficient: the management of haemoglobin SC disease
Author(s) -
Pecker Lydia H.,
Schaefer Beverly A.,
LuchtmanJones Lori
Publication year - 2017
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.14444
Subject(s) - disease , medicine , intensive care medicine , disease management , parkinson's disease
Summary Although haemoglobin SC (Hb SC ) 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 Hb SC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with Hb SC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (Hb SS ) patients. These studies are underpowered to allow definitive conclusions about Hb SC . We review the pathophysiology of Hb SC disease, including known and potential differences between Hb SS and Hb SC , and highlight knowledge gaps in Hb SC 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 Hb SC .