title: [Clinical and laboratory aspects of the Aspirin-like defect as hereditary thrombocytopathy].
abstract: The 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.
  
  
  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 <or= 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 displayed ALD. 4 family members were diagnosed with a mild ALD. Epistaxis, easy bruising, menorrhagia and perioperative hemorrhage were the most common bleeding symptoms, whereas three quarters of ALD patients presented with >or=1 bleeding symptoms.
  
  
  CONCLUSIONS
  In case of a bleeding tendency diagnostic procedures should rule out primary haemostatic defects. Hereditary platelet function defects including ALD are an important differential diagnosis. Family studies are reasonable.
authors.affiliation: Klinik und Poliklinik für Kinder- und Jugendmedizin, Bereich Pädiatrische Hämatologie und Onkologie, Universitätsklinikum Carl Gustav Carus, Technische Universität, 01307 Dresden.
authors.forename: N
authors.initials: N
authors.lastname: Rolf
authors.forename: P
authors.initials: P
authors.lastname: Bugert
authors.forename: S
authors.initials: S
authors.lastname: Gehrisch
authors.forename: G
authors.initials: G
authors.lastname: Siegert
authors.forename: M
authors.initials: M
authors.lastname: Suttorp
authors.forename: Ralf
authors.initials: R
authors.lastname: Knöfler
mesh_terms: D001241:Aspirin / Q000494:pharmacology*; D001778:Blood Coagulation Disorders / Q000209:etiology / Q000235:genetics; D001791:Blood Platelet Disorders / Q000175:diagnosis; D005190:Family; D005260:Female; D006474:Hemorrhagic Disorders / Q000209:etiology / Q000235:genetics; D006801:Humans; D008297:Male; D011453:Prostaglandins / Q000378:metabolism; D013921:Thrombocytopenia / Q000235:genetics*
chemical_list: D011453:Prostaglandins; D001241:Aspirin