Author Description

Sickle cell disease was one of the first diseases to be explained at the molecular level. In the years since the first edition of this book was published, further advances have been made, and much has been learned about the causes of variability in the natural history of the disease and of the reasons why there are such distinct variations in the patterns of morbidity and mortality. The role and importance of genetic modifiers such as alpha-thalassaemia and persisting high levels of fetal hemoglobin, and of the beta-globin haplotype, have been recognized and explored. Studies of the disease which compare geographically separated populations have contributed to understanding this variability. These findings have led to more rigorous thinking about the nature of the disease and a clearer appreciation of how to manage it. This new edition contains many new references and incorporates the latest thinking on the biology of the disease and the best practice in its management.