New advances in the pathophysiology and management of sickle cell disease.
The formation of the sickle cell hemoglobin polymer associated with deoxygenation of the sickle erythrocyte is a complex process. There are also many intracellular, extracellular, and erythrocyte membrane changes that are recognized to play important roles in the pathophysiology of this disease. The variability among these components accounts for the diversity observed in the phenotypic expression of sickle cell disease. This article reviews some of the recent developments in the understanding of the variables involved in the pathophysiology of sickle cell disease. Some of the new developments regarding clinical complications of sickle cell disease and their management are presented. New therapeutic options are reviewed. Finally, a discussion regarding transgenic models of sickle cell disease is presented.
Shafer
F E
FE
Department of Hematology/Oncology, Children's Hospital Oakland, CA 94609-1809, USA.
Vichinsky
E
E
D000755:Anemia, Sickle Cell / Q000503:physiopathology* / Q000628:therapy; D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic