[用因子VIII和因子IX制剂治疗血友病的并发症的矛盾出血]。

A H Sutor
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引用次数: 0

摘要

矛盾出血是血友病患者在接受替代治疗时因原发止血功能紊乱而发生的并发症。在长时间和高剂量的凝血因子- viii和凝血原复合物浓缩物治疗期间观察到它们。临床并发症,例如伤口愈合延迟以及皮肤和粘膜自发出血,在四分之一接受替代治疗的血友病患者中观察到。其中1 / 3的患者发现原发性止血病理参数(出血时间延长、潴留、收缩减少、ADP和胶原诱导的聚集、血小板因子3释放)。以下机制或物质可能是这些干扰的原因:纤维蛋白原和因子- viii分裂产物蛋白质含量高,主要是纤维蛋白原和因子viii相关抗原。抗原抗体反应Willebrand因子抑制剂的发展。对于异流性出血的治疗,已使用新鲜制备的冷沉淀,泼尼松和依坦。
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[Paradoxical bleeding as a complication of the treatment of hemophilia with factor VIII and factor IX preparations].

Paradoxical bleedings are complications occurring under replacement therapy in haemophiliacs by disturbancies of the primary haemostasis. They have been observed during treatment with factor-VIII- and prothrombin-complex concentrates of long duration and in high dosage. Clinical complications, for example delayed wound healing as well as spontaneous bleedings into the skin and from the mucous membranes, have been observed in one quarter of haemophiliacs under substitution therapy. In one third of these patients pathological parameters of primary haemostasis (prolonged bleeding time, reduced retention, retraction, ADP- and collagen-induced aggregation and the platelet factor 3 release) were found out. The following mechanisms or substances may be the cause for these disturbancies: 1. fibrinogen and factor-VIII split products 2. high content of proteins predominantly fibrinogen and factor-VIII-related antigen 3. antigen-antibody reactions 4. development of inhibitors against the Willebrand factor. For treatment of the paradoxical bleedings freshly prepared cryoprecipitate, prednison and Etamsylatum have been used.

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