Alleviating anaemia in patients with sickle cell disease (SCD) is crucial in managing acute complications, mitigating end-organ damage and preventing early mortality. Some disease-modifying and curative therapies have increased haemoglobin (Hb) levels to exceed 100 g/l, a threshold above which complications from red blood cell (RBC) transfusions have occurred, raising concern about whole-blood viscosity-related complications with these therapies. Here we discuss the rationale behind this limit, the effect of viscosity on blood flow and the applicability of this Hb threshold to therapies for SCD beyond RBC transfusions.
Keywords: blood transfusion; hydroxycarbamide; hyperviscosity; sickle cell disease; voxelotor.
© 2021 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.