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Diagnosis and management of neonatal alloimmune thrombocytopenia.
Transfusion Medicine Reviews ( IF 4.5 ) Pub Date : 2008-10-14 , DOI: 10.1016/j.tmrv.2008.05.003
Donald M Arnold 1 , James W Smith , John G Kelton
Affiliation  

Neonatal alloimmune thrombocytopenia (NAT) is a life-threatening bleeding disorder caused by maternal platelet antibodies produced in response to fetal platelet antigens inherited from the father. Antiplatelet antibodies cross the placenta and cause destruction of fetal platelets, leading to severe thrombocytopenia, and potentially bleeding, including fatal intracerebral hemorrhage. Incompatibilities between maternal and fetal platelets for the human platelet antigen 1a (previously called PL(A1)) account for most of the patients with NAT, but other antigens are commonly implicated. Diagnostic testing for NAT involves genotyping of maternal, paternal, and sometimes fetal DNA; platelet antigen phenotyping; and maternal platelet antibody investigations using specialized platelet glycoprotein specific assays. The management of women and infants at risk for NAT remains largely empiric; and mounting evidence points to prohibitive risks of invasive procedures such as fetal blood sampling and intrauterine platelet transfusions, except in rare circumstances. Improvements in our understanding of the pathophysiology of NAT, and of clinical and laboratory predictors of severity, may help develop better treatments and improve our ability to identify mothers at risk.

中文翻译:

新生儿同种免疫性血小板减少症的诊断和处理。

新生儿同种免疫血小板减少症(NAT)是一种威胁生命的出血性疾病,由母体血小板抗体引起,该抗体是针对父亲遗传的胎儿血小板抗原而产生的。抗血小板抗体穿过胎盘并引起胎儿血小板破坏,导致严重的血小板减少症,并可能出血,包括致命的脑内出血。人体血小板抗原1a(以前称为PL(A1))的孕妇血小板和胎儿血小板之间的不相容性是大多数NAT患者的原因,但通常涉及其他抗原。NAT的诊断测试涉及母体,父体,有时还有胎儿DNA的基因分型。血小板抗原表型 以及使用专门的血小板糖蛋白特异性测定法进行的孕妇血小板抗体研究。对患有NAT风险的妇女和婴儿的管理仍然十分经验;越来越多的证据表明,除极少数情况外,侵入性手术(如胎儿血液采样和子宫内血小板输注)的危险性很高。我们对NAT病理生理学以及严重程度的临床和实验室预测指标的了解的改善,可能有助于开发更好的治疗方法,并提高我们识别高危母亲的能力。
更新日期:2019-11-01
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