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

Abstract

UNLABELLED:

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.

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 <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.

CONCLUSION:

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: N Rolf, P Bugert, S Gehrisch, G Siegert, M Suttorp, Ralf Knöfler
• Journal: Hamostaseologie (Hamostaseologie) Vol. 29 Issue 2 Pg. 177-83 (May 2009) ISSN: 0720-9355 [Print] Germany
• Vernacular Title: Klinische und labordiagnostische Aspekte des Aspirin-like Defekts als hereditäre Thrombozytopathie.
• PMID: 19404514 (Publication Type: English Abstract, Journal Article)

Chemical References

• Prostaglandins
• Aspirin

Topics

• Aspirin (pharmacology)
• Blood Coagulation Disorders (etiology, genetics)
• Blood Platelet Disorders (diagnosis)
• Family
• Female
• Hemorrhagic Disorders (etiology, genetics)
• Humans
• Male
• Prostaglandins (metabolism)
• Thrombocytopenia (genetics)