Abstract

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.