The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease

Augusto B. Federici, Paolo Bucciarelli, Giancarlo Castaman, Maria G. Mazzucconi, Massimo Morfini, Angiola Rocino, Mario Schiavoni, Flora Peyvandi, Francesco Rodeghiero, Pier Mannuccio Mannucci

Research output: Contribution to journalArticlepeer-review

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 von Willebrand factor (VWF)/factor VIII concentrates. Among the 796 patients included, 75 (9.4%) needed treatment of 232 spontaneous bleeding events. BS >10 and VWF:ristocetin cofactor activity 10 remained highly associated in a multivariable Cox proportional hazard model (adjusted hazard ratio: 7.27 [95% confidence interval, 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. Therefore, BS can be considered a simple predictor of clinical outcomes of VWD and may identify patients needing intensive therapeutic regimens.

Original languageEnglish
Pages (from-to)4037-4044
Number of pages8
JournalBlood
Volume123
Issue number26
DOIs
Publication statusPublished - Jun 26 2014

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

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