IMEX   05356
INSTITUTO DE MEDICINA EXPERIMENTAL
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
DDAVP challenge test in patients with von Willebrand disease type 1 (VWD1) and low von Willebrand factor (LowVWF): analysis of 616 patients.
Autor/es:
ROMERO ML; BLANCO A; AGAZZONI M; WOODS AI; SANCHEZ LUCEROS, ANALIA; MESCHENGIESER SS; ALBERTO MF
Lugar:
Melbourne
Reunión:
Congreso; XXVII Congress of the International Society on Thrombosis and Haemostasis (ISTH); 2019
Institución organizadora:
International Society on Thrombosis and Haemostasis
Resumen:
Background: A DDAVP challenge test is usually recommended in patients with VWD1 and Low VWF to ensure that there is an adequate response in terms of VWF increase and to confirm the duration of VWF response. However, there is no standardized approach to test the biological response to DDAVP. (48)Aims: To evaluate the response of the VWF parameters and the eluglobulin lysis time (ELT) to the DDAVP challenge in retrospective cohort of VWD1 and Low VWF patients (according to updated ISTH criteria).Methods: 616 patients (VWD1: 62, Low VWF: 554; 21% ˂16yo) gave their informed consent for this study, which was approved by the institutional ethical committee. Good response to DDAVP (0.3 kg-1 body weight, intravenous) was defined as patients who, 1 and 2 hs after the end of the infusion, had an increase of plasma FVIII/VWF:RCo of at least 3-fold over baseline and reached levels at least 50 IU dL-1 for both values.Results: Demographic characteristics of population are described in table 1 and FVIII/VWF parameters and ELT at diagnosis/DDAVP test in children and adults are described in table 2.97% (596/616) showed good response to DDAVP. Eleven percent of patients (70/616) showed RCo/Ag