deamino-arginine-vasopressin and Amyloidosis

A 53-yr-old man with a 33-yr history of Crohn's ileocolitis, complicated by arthritis and cologastric fistulization, was diagnosed with GI amyloidosis at the time of proctocolectomy. He had marked proteinuria (4.2 g/24 h) and moderate renal insufficiency (BUN of 35 mg/dl and serum creatinine of 2.5 mg/dl). During the operation, he had severe bleeding that required 11 U of blood. Postoperatively, desmopressin was administered, which resulted in a prompt cessation of bleeding. This case demonstrates the efficacy of desmopressin in reversing the bleeding diathesis in surgical patients with amyloidosis complicated by renal insufficiency.

Topics: Amyloidosis; Blood Transfusion; Colectomy; Crohn Disease; Deamino Arginine Vasopressin; Disease Susceptibility; Follow-Up Studies; Hemostatics; Humans; Intraoperative Complications; Male; Middle Aged; Postoperative Hemorrhage; Renal Insufficiency; Treatment Outcome

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

Topics: Aged; Amyloidosis; Deamino Arginine Vasopressin; Gastrointestinal Hemorrhage; Humans; Male