Chai B C, Etches W S, Stewart M W, Siminoski K
University of Alberta, Edmonton, Canada.
Postgrad Med J. 1996 Feb;72(844):113-4. doi: 10.1136/pgmj.72.844.113.
We describe a man with adrenoleukodystrophy receiving Lorenzo's oil (glycerol trioleate and glycerol trierucate) who developed purpura, petechiae, and bleeding. Bleeding time was markedly increased (>20 min), although he had only borderline thrombocytopenia (120 x 10(9)/1) and conventional platelet aggregation studies were normal (except for a borderline response to low concentration collagen), as were results using a new technique employing immobilised von Willebrand factor. Together these results suggest that bleeding in this man resulted from a defect in vascular wall function or in the interaction of platelets with the endothelium.
我们描述了一名患有肾上腺脑白质营养不良症的男子,他在接受洛伦佐油(甘油三油酸酯和甘油三亚油酸酯)治疗时出现了紫癜、瘀点和出血症状。尽管他仅有临界性血小板减少症(120×10⁹/L),出血时间却显著延长(>20分钟),传统的血小板聚集试验结果正常(对低浓度胶原蛋白的反应仅为临界值除外),使用固定化血管性血友病因子的新技术检测结果也正常。这些结果共同表明,该男子的出血是由血管壁功能缺陷或血小板与内皮细胞相互作用异常所致。