Steinberg M H
Department of Medicine, University of Mississippi School of Medicine, Jackson.
J Clin Apher. 1991;6(4):221-3. doi: 10.1002/jca.2920060410.
Sickle cell anemia is present in about 1 in 600 Black Americans at birth. It claims the life of many infants, causes pain and suffering in adults, and usually culminates in premature death. We currently understand a great deal about this disease on a molecular, cellular, and clinical basis. The results of years of research are slowly being translated to innovative therapies and improvements in patient care. In this paper I review the pathophysiology of this disorder, highlight the currently available treatment, and discuss new forms of therapy that promise a more direct approach to avoiding the many complications of sickle cell anemia.
镰状细胞贫血在出生时约每600名美国黑人中就有1人患病。它夺走了许多婴儿的生命，给成年人带来痛苦，通常最终导致过早死亡。目前，我们在分子、细胞和临床层面上对这种疾病有了很多了解。多年的研究成果正逐渐转化为创新疗法和患者护理的改善。在本文中，我将回顾这种疾病的病理生理学，重点介绍目前可用的治疗方法，并讨论有望采用更直接方法避免镰状细胞贫血诸多并发症的新治疗形式。
