BIOLOGICAL AND CLINICAL RESPONSE TO DESMOPRESSIN IN CHILDREN WITH VON

SÁNCHEZ-LUCEROS A; MESCHENGIESER SS; TURDÓ K; WOODS AI; KEMPFER AC; BLANCO AN; LAZZARI MA

Suiza

Congreso; XXIst Congress of the International Society on Thrombosis and Haemostasis; 2007

International Society of Thrombosis and Haemostasis-Reach The World Travel Grant

Introduction: Since the first description in 1977, DDAVP has become widely used for the treatment of VWD. We reviewed the DDAVP challenge test and clinical response of DDAVP to prevent/treat bleeding in children with VWD. Methods: 231 children were included (mean age 10 years, range 2-16): type1:172, type2:30, probable:26, type3:3. Patients with hemophilia/VWD Normandy were excluded. Mean ISTH bleeding score was 2.6. Laboratory studies included: platelet count, platelet retention to glass beads (PR), bleeding time (BT), FVIII:C, VWF:Ag and VWF:RCo, VWF:RCo/Ag rate and VWF multimers. DDAVP (0.3 µg/kg) was given by I.V. saline infusion in 20 min. BT, PR, FVIII:C, VWF:Ag and VWF:RCo were tested before, 60 and 120 min post-DDAVP. If it not possible by age/weight, just one sample at 90 minutes post- DDAVP was taken (n= 19). Results: The response to DDAVP-test was: Good (ISTH): 148 patients; Good with partial response in BT/PR: 62; Incomplete (factor levels lower than 30%): 8; and Absent in 4 patients (type3: 3, type1: 1severe). Forty-six patients received DDAVP to prevent/treat bleeding: before surgery: 33, tooth extraction: 11, menorrhage: 6, epistaxis: 4 and muscle hematoma: 1. All showed good clinical response, including 16/46 children with partial response to the DDAVP-test. Another 8 children underwent surgery (6 ORL, 1 post-trauma) with preventive DDAVP infusion and a 2 years old boy, VWD type 1 severe, used DDAVP to stop profuse nose bleeding. Although these children had not been evaluated with a DDAVP-test before, all presented good clinical response. None serious adverse event was observed in any patient.    Conclusions: DDAVP is widely used for the treatment of VWD, as is affordable and it has no risk of blood-borne viruses. In children, bleeding score can not reflect the real bleeding risk, such as they had not sufficient time to face a risk situation. Incomplete response to DDAVP challenge test should not to censure the DDAVP use to prevent/stop bleeding in this population. Reach The World Travel Grant.  Reach The World Travel Grant.  Reach The World Travel Grant.