Analyses of the bleeding tendency by means of the bleeding score (BS) have been proposed until now to confirm diagnosis but not to predict clinical outcomes in patients with inherited von Willebrand disease (VWD). We prospectively followed up for 1 year 796 Italian patients with different types of VWD to determine whether the previous BS of European VWD1 is useful to predict the occurrence of spontaneous bleeds severe enough to require replacement therapy with desmopressin (DDAVP) and/or VWF/FVIII concentrates. Among the 796 patients included, 75 (9.4%) needed treatment for 232 spontaneous bleeding events. BS>10 and VWF:RCo<10 U/dL were associated with the risk of bleeding, but only a BS>10 remained highly associated in a multivariable Cox proportional hazard model (adjusted hazard ratio: 7.27 (95% confidence intervals: 3.83-13.83). Although the bleeding event-free survival was different in VWD types, only a BS>10 could predict for each type which patient had bleeding events severe enough to require treatment with DDAVP and/or concentrates. This prospective study shows that VWD patients characterized by BS>10 are more exposed than others to frequent treatments for spontaneous bleeds. Therefore, BS can be considered a simple predictor of clinical outcomes of VWD and may identify patients needing intensive therapeutic regimens
The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease: a prospective cohort study of 796 cases / Federici, Ab; Bucciarelli, P; Castaman, G; Mazzucconi, Maria Gabriella; Morfini, M; Rocino, A; Schiavoni, M; Peyvandi, F; Rodeghiero, F; Mannucci, P. M.. - In: BLOOD. - ISSN 0006-4971. - STAMPA. - 123:26(2014), pp. 4037-4044. [10.1182/blood-2014-02-557264]
The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease: a prospective cohort study of 796 cases.
MAZZUCCONI, Maria Gabriella;
2014
Abstract
Analyses of the bleeding tendency by means of the bleeding score (BS) have been proposed until now to confirm diagnosis but not to predict clinical outcomes in patients with inherited von Willebrand disease (VWD). We prospectively followed up for 1 year 796 Italian patients with different types of VWD to determine whether the previous BS of European VWD1 is useful to predict the occurrence of spontaneous bleeds severe enough to require replacement therapy with desmopressin (DDAVP) and/or VWF/FVIII concentrates. Among the 796 patients included, 75 (9.4%) needed treatment for 232 spontaneous bleeding events. BS>10 and VWF:RCo<10 U/dL were associated with the risk of bleeding, but only a BS>10 remained highly associated in a multivariable Cox proportional hazard model (adjusted hazard ratio: 7.27 (95% confidence intervals: 3.83-13.83). Although the bleeding event-free survival was different in VWD types, only a BS>10 could predict for each type which patient had bleeding events severe enough to require treatment with DDAVP and/or concentrates. This prospective study shows that VWD patients characterized by BS>10 are more exposed than others to frequent treatments for spontaneous bleeds. Therefore, BS can be considered a simple predictor of clinical outcomes of VWD and may identify patients needing intensive therapeutic regimensI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.