[Aspirin-like defect - a hereditary thrombocytopathy due to impaired release of platelet adenosine diphosphate].

Abstract

This report presents the case of a 36 year old woman with an inborn haemorrhagic syndrome, who exhibited a lifelong history of spontaneous bruising, nose bleeding, prolonged bleeding after tooth extraction, and menometrorrhagia. The routine tests of haemostasis were consistent with impaired platelet functions. The diagnosis of the "Aspirin-like defect" was made on the basis of the following findings: the bleeding time was prolonged, whereas the platelet count and morphology were normal; platelet retention in glass bead filters was unmeasurable. ADP-induced platelet aggregation was normal, while it was markedly reduced with collagen and epinephrine. The platelet ADP and ATP content, as well as the ATP/ADP ratio were within normal limits. Aggregation of platelets pre-incubated with aspirin was only slightly reduced when induced by ADP, collagen, or epinephrine. These findings suggest that the thrombocytopathy in our patient is due to an impaired ADP release from the platelet granules containing normal quantities of adenine nucleotides. A similar disorder is observed in normal subjects after aspirin ingestion, and therefore the defect described in this paper is referred to as the "Aspirin-like" defect.