Haematological and clinical aspects of sickle cell disease in Britain

Abstract

Publisher Summary This chapter focuses on the hematological and clinical aspects of sickle cell disease (SCD) in Britain. SCD was considered to be rare and of little significance in Britain. The great majority of cases in the United Kingdom are British born of West Indian or African extraction and are permanently domiciled in Britain. About 5% are temporarily resident in the United Kingdom as students, visitors, diplomats, and businessmen from Africa, and some 10% are their children. Most cases are under the age of 30, and over 20% live in the boroughs of Brent and Lambeth in London and in Birmingham, the remainder resides in other London boroughs, the Midlands, and Mersey side. In Brent, almost all 26 pregnancies reviewed in 1984 suffered some SCD complication. These included a maternal death from stroke at 39 weeks, a stillbirth following a fulminant abdominal sickling crisis at 29 weeks, splenic infarction in the second and third trimesters and rapidly progressive aseptic necrosis of the hip and sickle retinopathy. An important feature was the postpartum cluster of pulmonary thromboembolic events in patients with SC which had been abolished by the use of prophylactic twice daily subcutaneous heparin from 36 weeks to 6 weeks postpartum. Adequate hypertransfusion can prevent some of these complications if started sufficiently early in pregnancy.