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血管性血友病的免疫学研究:血管性血友病患者输注血浆浓缩物后VIII因子/血管性血友病蛋白的改变

Immunologic studies in von Willebrand's disease: alteration of factor VIII/von Willebrand protein after transfusion with plasma concentrates in patients with von Willebrand's disease.

作者信息

Sultan Y, Simeon J, Maisonneuve P, Caen J P

出版信息

Thromb Haemost. 1976 Feb 29;35(1):110-9.

PMID:1085498
Abstract

Two patients with a severe von Willebrand's disease characterized by no detectable factor VIII related antigen in their plasma received transfusions of cryoprecipitate. The bleeding time was corrected for a short period of time and returned to its pretransfusional value although the other parameters of the disease were still corrected. Electrophoretic and immunologic properties of factor VIII related antigen infused were determined serially after transfusion. Modifications of these properties occurred progressively after transfusion. The half disappearance time of F. VIII R.A. was determined and found to be considerably shorter than in hemophilic recipients. This study suggests an alteration in vivo of F. VIII R.A. infused into von Willebrand recipients.

摘要

两名患有严重血管性血友病的患者,其血浆中检测不到因子VIII相关抗原,接受了冷沉淀输血。出血时间在短时间内得到纠正,但随后又恢复到输血前的值,尽管该疾病的其他参数仍得到纠正。输血后连续测定所输注的因子VIII相关抗原的电泳和免疫学特性。这些特性在输血后逐渐发生改变。测定了因子VIII相关抗原(F. VIII R.A.)的半衰期,发现其比血友病受血者的半衰期短得多。这项研究表明,输入血管性血友病受血者体内的F. VIII R.A. 发生了改变。

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