Takahashi H, Tatewaki W, Nagayama R, Hanano M, Tamura M, Yamaguchi T, Takizawa S, Wada K, Shibata A
First Department of Internal Medicine, Niigata University School of Medicine, Japan.
Haemostasis. 1987;17(6):353-60. doi: 10.1159/000215770.
Cryoprecipitate has proved to correct the hemostatic defects in von Willebrand's disease (vWD) and platelet-type vWD. However, recent studies have revealed that transmission of the AIDS retrovirus (HIV) occurs through exposure to blood products including cryoprecipitate. Treatment with heat-treated factor VIII/von Willebrand factor (vWf) concentrates may have certain advantages over treatment with nonheated products, if these preparations are efficacious in these disorders. We found that a commercially available factor VIII/vWf concentrate, Haemate P, contained the high-molecular-weight multimers of vWf and had a ratio of ristocetin cofactor (RCof) to vWf antigen (vWf:Ag) close to unity. In addition, its capacity to directly induce aggregation of platelet-type vWD platelets in vitro was similar to that for cryoprecipitate. When infused into a patient with platelet-type vWD, Haemate P shortened the prolonged bleeding time and caused spontaneous platelet aggregation in vitro with a mild diminution of platelet count. These results indicate that some of the heat-treated factor VIII/vWf concentrates may provide a safer, yet still effective, treatment for platelet-type vWD.
冷沉淀已被证明可纠正血管性血友病（vWD）和血小板型vWD的止血缺陷。然而，最近的研究表明，艾滋病逆转录病毒（HIV）可通过接触包括冷沉淀在内的血液制品传播。如果热处理的凝血因子VIII/血管性血友病因子（vWf）浓缩物在这些疾病中有效，那么用其治疗可能比用未加热的制品治疗具有某些优势。我们发现，一种市售的凝血因子VIII/vWf浓缩物Haemate P含有高分子量的vWf多聚体，其瑞斯托霉素辅因子（RCof）与vWf抗原（vWf:Ag）的比率接近1。此外，它在体外直接诱导血小板型vWD血小板聚集的能力与冷沉淀相似。当将Haemate P输注给一名血小板型vWD患者时，它缩短了延长的出血时间，并在体外引起自发血小板聚集，同时血小板计数略有减少。这些结果表明，一些热处理的凝血因子VIII/vWf浓缩物可能为血小板型vWD提供一种更安全但仍有效的治疗方法。
