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[新生儿同种免疫性血小板减少症]

[Neonatal alloimmune thrombopenia].

作者信息

Muller J Y, Patereau C, Reznikoff-Etievant M F, Kaplan C, Simonney N

出版信息

Rev Fr Transfus Immunohematol. 1985 Dec;28(6):625-41. doi: 10.1016/s0338-4535(85)80007-6.

Abstract

88 families in which 84 cases of neonatal alloimmune thrombocytopenia (NAT) occurred, were studied. In 84 families, the NAT was the consequence of an incompatibility in the PLA system. Furthermore, the phenotype HLA-DR3 increases greatly the risk of immunisation (RR: 76,5). The importance of the risk of neurological sequellae was shown by the clinical study (about 25% of the surviving neonates). The occurrence of the accident at the first birth of a PLA1 positive child in a sibship was frequent (59%). In addition, the NAT recurred at each birth of a PLA1 positive child with only five exceptions. All of them concern a female neonate and this might be meaningful. Therapeutical data are heterogeneous and difficult to interpret. However, it appears that the prevention of obstetrical traumatism by caesarean section and compatible platelet transfusions are useful. It is too early to evaluate the efficacy of prenatal transfusions of mother's washed platelets. However, in the two cases in which we use them, they gave a good and sustained platelet count increment. The prenatal diagnosis of NAT and the PLA grouping of the foetus has been proposed in three cases and are feasible at 20 weeks of pregnancy.

摘要

对88个家庭进行了研究,这些家庭中有84例新生儿同种免疫性血小板减少症(NAT)发生。在84个家庭中,NAT是PLA系统不相容的结果。此外,HLA - DR3表型大大增加了免疫风险(相对危险度:76.5)。临床研究显示了神经后遗症风险的重要性(约25%的存活新生儿)。在同胞关系中,PLA1阳性儿童首次出生时发生该病症的情况很常见(59%)。此外,除了五个例外情况,PLA1阳性儿童每次出生时NAT都会复发。所有这些例外都涉及女新生儿,这可能有意义。治疗数据参差不齐且难以解释。然而,剖宫产和输注相容血小板预防产科创伤似乎是有用的。评估母亲洗涤血小板产前输注的疗效还为时过早。然而,在我们使用它们的两例中,血小板计数有良好且持续的增加。已在三例中提出NAT的产前诊断和胎儿的PLA分型,在妊娠20周时是可行的。

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