Ballas S K, Mohandas N
Sickle Cell Center, Cardeza Foundation for Hematologic Research, Philadelphia, Pennsylvania, USA.
Hematol Oncol Clin North Am. 1996 Dec;10(6):1221-39. doi: 10.1016/s0889-8588(05)70396-8.
Sickle cell anemia is a very complex disease. Vascular occlusion, the major event that accompanies SS, is itself a complex and multifactional process. Microvascular occlusion results in acute painful crises, whereas macrovascular occlusion seems to be the cause of organ failure. Understanding the basic pathophysiologic events of vascular occlusion may elucidate the clinical manifestations of SS, its natural history, and complications, and it may give new insights into preventive and curative therapy.
镰状细胞贫血是一种非常复杂的疾病。血管闭塞是镰状细胞贫血(SS)伴随的主要事件,其本身就是一个复杂且多因素的过程。微血管闭塞会导致急性疼痛性危机,而大血管闭塞似乎是器官衰竭的原因。了解血管闭塞的基本病理生理事件可能有助于阐明镰状细胞贫血的临床表现、其自然病程和并发症,并且可能为预防和治疗提供新的见解。