Platelet-type von Willebrand's disease: characterization of a new bleeding disorder
Platelet-type von Willebrand's disease: characterization of a new bleeding disorder
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血小板型血管性血友病：一种新出血性疾病的特征
DOI：
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发表时间：
1982
期刊：
影响因子：
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通讯作者：
A. Castella
中科院分区：
文献类型：
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作者：
Jl Miller;A. Castella
一个常染色体传播的出血素质共享一些，但不是全部，以前描述的功能在血管性血友病（vWd）进行了研究，在五名患者代表三代一个家庭。出血时间在正常上限范围内，同时血小板计数正常偏低，凝血因子VIII凝血活性和VIII相关抗原正常，VIII-瑞斯托康辅因子活性降低，高分子量凝血因子VIII/血管性血友病因子（VIII/vWF）多聚体选择性降低，以及在低瑞斯托康浓度下瑞斯托康诱导的血小板凝集增加是特征性的。患者VIII/vWF与洗涤的正常血小板的结合在正常范围内，而正常VIII/vWF与患者血小板的结合显著增加（0.6 mg/ml利托那肽时p <0.001）。因此，这种疾病似乎涉及影响血小板-VIII/vWF相互作用的内在血小板异常。有人建议，vWD的概念应扩大到包括这种异常的患者，这可能是适当的称为“血小板型血管性血友病”。
An autosomally transmitted bleeding diathesis sharing some, but not all, features previously described in von Willebrand's disease (vWd) was studied in five patients representing three generations of a single family. Bleeding times in the upper normal range in conjunction with low-normal platelet counts, normal factor VIII coagulant activity and VIII-related antigen, decreased VIII-ristocetin cofactor activity, selective decrease of the higher molecule weight factor VIII/von Willebrand factor (VIII/vWF) multimers, and increased ristocetin- induced platelet agglutination at low ristocetin concentrations were characteristic. Binding of patient VIII/vWF to washed normal platelets was within normal limits, whereas binding of normal VIII/vWF to patient platelets was significantly increased (p less than 0.001 at 0.6 mg/ml ristocetin). This disorder accordingly appears to involve an intrinsic platelet abnormality affecting platelet-VIII/vWF interactions. It is proposed that the concept of vWD be broadened to include patients with this abnormality, which may appropriately be called “Platelet-type von Willebrand's disease.”