BSH 2021 | Determinants of sickle cell disease severity
David Rees, MA, MBBS, FRCP, FRCPath, FRCPCH, King’s College Hospital, London, UK, explains the reasoning behind the varying severities of sickle cell disease (SCD) that exist, highlighting genetic and environmental factors. Coinheriting thalassemia and genes that increase fetal hemoglobin (HbF) can reduce the severity of SCD, and gene therapies are being developed that can repress BCL11A to increase HbF expression. Environmental factors affecting SCD severity are alternatively less well studied, but Prof. Rees suggests they play an important part; healthcare access, prevalence of malaria and exposure to cold temperatures can affect SCD severity. This interview took place at the 2021 British Society for Haematology Annual Scientific Meeting.
