deamino-arginine-vasopressin and Familial-Mediterranean-Fever

Systemic amyloidosis may often be complicated with haemorrhagic tendency. The causes of this manifestation are factor deficiencies, hyperfibrinolysis and vasculopathy. In order to investigate the role of platelets, if any, we performed platelet aggregation tests with different aggregants in 10 patients with systemic amyloidosis due to familial Mediterranean fever and 10 healthy controls. Platelet aggregation was defective with different aggregants (ADP, epinephrine, collagen) in patients compared with controls. Platelet aggregation tests repeated after desmopressin (DDAVP) administration were normalized. These findings may suggest a role of a platelet aggregation defect in haemorrhagic diathesis complicating systemic amyloidosis. DDAVP may benefit patients with this disease in case of bleeding and before surgical interventions.

Topics: Adenosine Diphosphate; Adolescent; Adult; Amyloidosis; Collagen; Deamino Arginine Vasopressin; Epinephrine; Familial Mediterranean Fever; Female; Humans; In Vitro Techniques; Male; Platelet Aggregation; Reference Values