作者: G. CASTAMAN , A. B. FEDERICI , A. TOSETTO , S. LA MARCA , F. STUFANO
DOI: 10.1111/J.1538-7836.2012.04661.X
关键词:
摘要: Summary. Background: Type 2A and 2M von Willebrand disease (VWD2A VWD2M) are characterized by the presence of a dysfunctional factor (VWF) variable bleeding tendency. So far, head-to-head comparison clinical history risk between VWD2A VWD2M has never been provided in prospective manner. Aim study: We assessed incidence rate characteristics two cohorts 17 families (46 patients) with 15 (61 prospectively followed-up for 24 months. VWF gene mutations were all them. Results: Mean score (BS) antigen at enrollment significantly higher patients (P = 0.007). No correlation activity or VIII levels severity BS was observed. The spontaneous requiring treatment 107/100 patient-years (95% CI, 88.3–131) compared 40/100 30–53) (P < 0.001). BS ≥ 10 those 0–2. Furthermore, 54 episodes gastrointestinal occurred 17/46 (36.9%) seven 2/61 (3.3%) (P < 0.0001). Conclusion: Bleeding tendency is greater than that VWD2M, not explained mainly due to an increased bleeding.