Shafer F E, Vichinsky E
Department of Hematology/Oncology, Children's Hospital Oakland, CA 94609-1809, USA.
Curr Opin Hematol. 1994 Mar;1(2):125-35.
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.
与镰状红细胞脱氧相关的镰状细胞血红蛋白聚合物的形成是一个复杂的过程。还有许多细胞内、细胞外和红细胞膜的变化，这些变化在该疾病的病理生理学中起着重要作用。这些成分之间的变异性解释了镰状细胞病表型表达中观察到的多样性。本文综述了在理解镰状细胞病病理生理学中涉及的变量方面的一些最新进展。介绍了关于镰状细胞病临床并发症及其管理的一些新进展。综述了新的治疗选择。最后，讨论了镰状细胞病的转基因模型。
