Vadasz Brian, Chen Pingguo, Yougbaré Issaka, Zdravic Darko, Li June, Li Conglei, Carrim Naadiya, Ni Heyu
Toronto Platelet Immunobiology Group, Toronto, ON, Canada; Department of Laboratory Medicine, Keenan Research Centre for Biomedical Science, St. Michael's Hospital, Toronto, ON, Canada; Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.
Toronto Platelet Immunobiology Group, Toronto, ON, Canada; Department of Laboratory Medicine, Keenan Research Centre for Biomedical Science, St. Michael's Hospital, Toronto, ON, Canada; Canadian Blood Services, Toronto, ON, Canada.
Genes Dis. 2015 Jun 1;2(2):173-185. doi: 10.1016/j.gendis.2015.02.003.
Platelets play critical roles in hemostasis and thrombosis. Emerging evidence indicates that they are versatile cells and also involved in many other physiological processes and disease states. Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a life threatening bleeding disorder caused by fetal platelet destruction by maternal alloantibodies developed during pregnancy. Gene polymorphisms cause platelet surface protein incompatibilities between mother and fetus, and ultimately lead to maternal alloimmunization. FNAIT is the most common cause of intracranial hemorrhage in full-term infants and can also lead to intrauterine growth retardation and miscarriage. Proper diagnosis, prevention and treatment of FNAIT is challenging due to insufficient knowledge of the disease and a lack of routine screening as well as its frequent occurrence in first pregnancies. Given the ethical difficulties in performing basic research on human fetuses and neonates, animal models are essential to improve our understanding of the pathogenesis and treatment of FNAIT. The aim of this review is to provide an overview on platelets, hemostasis and thrombocytopenia with a focus on the advancements made in FNAIT by utilizing animal models.
血小板在止血和血栓形成中发挥着关键作用。新出现的证据表明,它们是多功能细胞,还参与许多其他生理过程和疾病状态。胎儿和新生儿同种免疫性血小板减少症(FNAIT)是一种危及生命的出血性疾病,由孕期母体产生的同种抗体破坏胎儿血小板所致。基因多态性导致母体与胎儿之间血小板表面蛋白不相容,最终导致母体同种免疫。FNAIT是足月婴儿颅内出血的最常见原因,还可导致宫内生长受限和流产。由于对该疾病了解不足、缺乏常规筛查以及其在初产妇中频繁发生,FNAIT的正确诊断、预防和治疗具有挑战性。鉴于对人类胎儿和新生儿进行基础研究存在伦理困难,动物模型对于增进我们对FNAIT发病机制和治疗的理解至关重要。本综述的目的是概述血小板、止血和血小板减少症,重点关注利用动物模型在FNAIT方面取得的进展。