Rezaei Kalantari H
Universitè de Liège, Service d'Hématologie.
Rev Med Liege. 2001 Oct;56(10):671-5.
Vaso-occlusive crisis, thromboembolic events and acute chest syndrome are the most frequent complications in patients with sickle cell disease. Appropriate management of the vaso-occlusive painful crisis includes aggressive analgesia and hydratation. For thromboembolic events, anticoagulation and acetylsalicylic acid with dipyridamole may be considered. Transfusion has a place during some crisis, when hemolysis is increased. Hydroxyurea, a drug that induce foetal hemoglobin synthesis until 30%, has a therapeutic benefit in decreasing the number of vaso-occlusive episodes, of transfusions and that of the acute chest syndrome. Currently, the only treatment that allows definitive cures is familial allogeneic stem cell transplantation. New agents are currently under clinical evaluation such as NO and arginine for aggressive crisis or other aggressive complications and administration magnesium and clotrimazole to prevent intracellular deshydratation. These drugs block cation-transport channels in erythrocyte membranes.
血管闭塞性危机、血栓栓塞事件和急性胸部综合征是镰状细胞病患者最常见的并发症。对血管闭塞性疼痛危机的适当管理包括积极镇痛和补液。对于血栓栓塞事件,可考虑抗凝治疗以及使用乙酰水杨酸联合双嘧达莫。在某些危机期间,当溶血增加时,输血有一定作用。羟基脲是一种可诱导胎儿血红蛋白合成达30%的药物,在减少血管闭塞发作次数、输血次数以及急性胸部综合征发作次数方面具有治疗益处。目前,唯一能实现根治的治疗方法是家族性异基因干细胞移植。目前新药物正在进行临床评估,如用于治疗严重危机或其他严重并发症的一氧化氮和精氨酸,以及用于预防细胞内脱水的镁和克霉唑。这些药物可阻断红细胞膜上的阳离子转运通道。