Clinical and laboratory aspects of the Aspirin-like defect as hereditary thrombocytopathy

SummaryThe Aspirin-like defect (ALD) is caused by defects in the intraplatelet arachidonic acid (AA)-metabolism. We here present the characteristics of a larger cohort in a single centre. Patients, methods: Based on 17 ALD index patients bleeding symptoms, agonist-induced platelet aggregation and closure times in the PFA-100® test were analysed in a family cohort of altogether 52 individuals from 17 families. Absent aggregation to AA (maximal aggregation ≤10%) was the main diagnostic criterion. A mild ALD was diagnosed when aggregation was 11–40%. Results: In addition to 17 ALD index patients, 13 family members…