PLATELET-AGGREGATION INDUCED BY 1-DESAMINO-8-D-ARGININE VASOPRESSIN (DDAVP) IN TYPE-IIB VONWILLEBRANDS DISEASE
PLATELET-AGGREGATION INDUCED BY 1-DESAMINO-8-D-ARGININE VASOPRESSIN (DDAVP) IN TYPE-IIB VONWILLEBRANDS DISEASE
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DOI：
10.1056/nejm198310063091402
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发表时间：
1983-01-01
影响因子：
158.5
通讯作者：
SJORIN, E
中科院分区：
文献类型：
--
作者：
HOLMBERG, L;NILSSON, IM;SJORIN, E
IIB 型冯维勒布兰德病是这种疾病的一种独特形式，其中血浆中存在异常的因子 VIII 冯维勒布兰德因子多聚体（但血小板中存在正常多聚体），并且在瑞斯托菌素存在的情况下，冯维勒布兰德因子与血小板之间的相互作用增强。输注醋酸去氨加压素（1-去氨-8-D-精氨酸加压素 [DDAVP]）是一种用于治疗冯·维勒布兰德氏病的药物，会导致 IIB 型疾病患者出现血小板聚集和血小板减少。在体外，正常血浆中的血小板以及在输注 DDAVP 之前从 IIB 型疾病患者获得的血小板在加入接受 DDAVP 治疗的 IIB 型患者的贫血小板血浆后发生聚集。血小板聚集与因子 VIII/血管性血友病因子多聚体吸附到血小板上有关，并被 EDTA 抑制。在IIB型冯维勒布兰德氏病中，DDAVP显然会释放一种具有血小板聚集特性的异常因子。 DDAVP 不应用于治疗 IIB 型疾病患者，因为循环中血小板聚集体的存在可能有害。
Type IIB von Willebrand''s disease is a distinct form of this disorder, in which there are abnormal factor VIII von Willebrand factor multimers in plasma (but normal multimers in platelets) and heightened interaction between the von Willebrand factor and platelets in the presence of ristocetin. Infusion of desmopressin acetate (1-desamino-8-D-arginine vasopressin [DDAVP]), an agent used in the treatment of von Willebrand''s disease, causes platelet aggregation and thrombocytopenia in patients with Type IIB disease. In vitro, platelets in normal plasma and those obtained from patients with Type IIB disease before DDAVP infusion aggregated upon the addition of platelet-poor plasma from Type IIB patients treated with DDAVP. Platelet aggregation was associated with adsorption of multimers of factor VIII/von Willebrand factor onto the platelets and was inhibited by EDTA. In Type IIB von Willebrand''s disease, DDAVP apparently releases an abnormal factor with platelet-aggregating properties. DDAVP should not be used to treat patients with Type IIB disease, since the presence of platelet aggregates in the circulation may be harmful.