Sickle cell disease is one of the grand diseases in the terminology of Victorian pathology, because its widespread and multitudinous manifestations may affect any organ system in the body. It has served as a model of molecular disease being one of the first genetic disorders to be explained at the molecular level. Since publication of the second edition, however, further advances have been made. Much has been learnt about the causes of variability in the natural history of the disease and of the reasons why there are such marked variations in the patterns of morbidity and mortality. As...