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Different bleeding risk in type 2A and 2M von Willebrand disease: a 2‐year prospective study in 107 patients
Author(s) -
CASTAMAN G.,
FEDERICI A. B.,
TOSETTO A.,
LA MARCA S.,
STUFANO F.,
MANNUCCI P. M.,
RODEGHIERO F.
Publication year - 2012
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/j.1538-7836.2012.04661.x
Subject(s) - medicine , von willebrand factor , von willebrand disease , gastroenterology , incidence (geometry) , prospective cohort study , gastrointestinal bleeding , risk factor , surgery , platelet , physics , optics
Summary. Background: Type 2A and 2M von Willebrand disease (VWD2A and VWD2M) are characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. So far, a head‐to‐head comparison of the clinical history and bleeding risk between VWD2A and VWD2M has never been provided in a prospective manner. Aim of the study: We assessed the bleeding incidence rate and clinical characteristics in two cohorts of 17 families (46 patients) with VWD2A and 15 families (61 patients) with VWD2M prospectively followed‐up for 24 months. VWF gene mutations were characterized in all of them. Results: Mean bleeding score (BS) and VWF antigen at enrollment were significantly higher in VWD2A patients ( P = 0.007). No correlation between VWF activity or factor VIII levels and the severity of BS was observed. The incidence rate of spontaneous bleeding requiring treatment was 107/100 patient‐years (95% CI, 88.3–131) in VWD2A compared with 40/100 patient‐years (95% CI, 30–53) in VWD2M ( P < 0.001). The risk of bleeding was significantly higher in patients with BS ≥ 10 at enrollment compared with those with BS 0–2. Furthermore, 54 episodes of gastrointestinal bleeding occurred in 17/46 (36.9%) VWD2A patients and seven in 2/61 (3.3%) VWD2M patients ( P < 0.0001). Conclusion: Bleeding tendency in VWD2A is greater than that of VWD2M, is not explained by factor VIII or VWF levels and is mainly due to an increased incidence of gastrointestinal bleeding.