EFFICACY AND SAFETY OF DESMOPRESSIN (DDAVP) AS A HAEMOSTATIC AGENT IN MILD HEMOPHILIA AND TYPE I, IIA

Desmopressin is a synthetic analog of antiduretic hormone used for control and treatment of noctornal enurisis in patients with diabetes insipitus. Since 1977, desmopressin was shown to useful in prevention and treatment of bleedings in patients with mild hemophilia and vWD.This non transfusional haemostatic agent when infused interavenously, is expected to increase transiently F VIIIc and vWF, 3-5 times above the basal, levels, within 30-60 min and thereby corrects both aPTT and BT defects. The mode of action is only partially understood.With the aim to survey DDAVP haemostatic effect and to avoid the use of blood products to control or to prevent of bleeding episodes we used desmopressin in 30 Iranian patients with mild hemophilia and vWD (type 1, 2A)Desmopressin (Emosint-Kedrion-Italy) was administered intravenously, in a dose of 0.3 g/kg body weight, diluted in 50 ml normal saline and infused over 30 min. From all patients, 5-7 ml blood sample was taken before and 30-60 min after DDAVP infusion. For evaluation of coagulation, F VIIIc was measured by a one-stage clotting time method, vWF: Ag by Elisa and vWF activity by vWF R. CoF activity, and BT by IVY method.The results showed that clotting assays in 20 patients with mild hemophilia A (F VIII, 5-30%) 30-60 min after DDAVP infusion showed increasing of F VIIIc median 3.7 times above the basal level and aPTT corrected median 13 s, without any important side effects.This treatment in 10 patients with vWD (type 1, 2A) increased F VIIIc median 3.7 and vWF, 3 times above the basal levels, leading to shorting of aPTT 12.9 s and BT, 3.4 min in compare to pre treatment levels.Our results showed that desmopressin in mild hemophilia and vWD (type 1 & 2A) is effective and safe, because it provide a form of autologous replacement therapy and usually permits the avoidance of using clotting factor and with potential of decreasing in the cost of treatment.