Manwani Deepa, Frenette Paul S
Departments of 1Pediatrics.
Hematology Am Soc Hematol Educ Program. 2013;2013:362-9. doi: 10.1182/asheducation-2013.1.362.
Recurrent and unpredictable episodes of vaso-occlusion are the hallmark of sickle cell disease. Symptomatic management and prevention of these events using the fetal hemoglobin-reactivating agent hydroxyurea are currently the mainstay of treatment. Discoveries over the past 2 decades have highlighted the important contributions of various cellular and soluble participants in the vaso-occlusive cascade. The role of these elements and the opportunities for therapeutic intervention are summarized in this review.
血管阻塞反复发作且不可预测是镰状细胞病的标志。使用胎儿血红蛋白激活剂羟基脲对这些事件进行症状管理和预防是目前的主要治疗方法。过去20年的研究发现突出了各种细胞和可溶性参与成分在血管阻塞级联反应中的重要作用。本文综述总结了这些成分的作用以及治疗干预的机会。
