Owen C A, Goldstein N P, Bowie J W
Arch Intern Med. 1976 Feb;136(2):148-52.
Sixteen patients with Wilson disease (hepatolenticular degeneration) were studied from the hemostatic point of view, particularly with regard to platelet function. Five of the patients had a mild bleeding tendency that was characterized by easy bruising. Moderate thrombocytopenia was observed in three of the five bleeders and in two of the others. One bleeder was thrombocytotic and hyperfibrinogenemic. Bleeding times, platelet retention and prothrombin consumption were abnormal rarely. However, 15 of the 16 patients had some abnormality of platelet aggregation: one when adenosine diphosphate was added to platelet rich plasma, three when epinephrine was added, and the remainder when collagen was added. The collagen abnormalities were delayed or absent aggregation (five patients, four of whom were bleeders) and absence of a change of shape (12 of the 16 patients). Platelet aggregation was completely normal in only one patient.
从止血角度，尤其是血小板功能方面，对16例肝豆状核变性（威尔逊病）患者进行了研究。其中5例患者有轻度出血倾向，表现为容易出现瘀伤。在这5例有出血倾向的患者中有3例以及另外2例患者观察到中度血小板减少。1例有出血倾向的患者血小板增多且纤维蛋白原血症。出血时间、血小板滞留和凝血酶原消耗很少出现异常。然而，16例患者中有15例存在某种血小板聚集异常：1例在向富含血小板血浆中添加二磷酸腺苷时出现异常，3例在添加肾上腺素时出现异常，其余患者在添加胶原蛋白时出现异常。胶原蛋白导致的异常包括聚集延迟或无聚集（5例患者，其中4例有出血倾向）以及形状无变化（16例患者中的12例）。只有1例患者的血小板聚集完全正常。
