Search results for "von Willebrand disease"

A systematic overview of the first pasteurised VWF/FVIII medicinal product, Haemate P/Humate -P: history and clinical performance

2008

Patients with von Willebrand disease (VWD) and haemophilia A (HA) lack, to varying degrees, the von Willebrand factor (VWF) and coagulation factor VIII (FVIII) that are critical for normal haemostasis. These conditions in turn make patients prone to uncontrolled bleeding. Historically, patients with severe forms of VWD or HA were crippled before adulthood and their life expectancy was significantly reduced. Over the past decades, specific coagulation factor replacement therapies including Haemate P, have been developed to help patients achieve and maintain normal haemostasis. Haemate P is a human, plasma-derived VWF/FVIII medicinal product, which was first licensed in Germany in 1981 for th…

Results of a Prospective, Open-Label Trial to Assess the Efficacy, Safety and Immunogenicity of Wilate® in Children Under 6 Years of Age with Inherit…

2011

Abstract Abstract 3330 Introduction: Wilate® is a new-generation plasma-derived concentrate of von Willebrand factor (VWF) and factor VIII (FVIII) developed for treatment of patients with von Willebrand disease (VWD) and haemophilia A. The objective of this study was to assess efficacy, safety and immunogenicity of Wilate in children below 6 years of age with inherited VWD. Methods: The prospective, multi-centre trial included patients with inherited VWD of any type below 6 years of age with insufficient response to DDAVP. Any clinical event requiring VWF/FVIII concentrate treatment within 1 year was treated with Wilate. Efficacy was recorded by using a 4-point VRS and in vivo recovery of F…

Small bowel angiodysplasia associated with von Willebrand's disease: report of a case.

2006

The association between angiodysplasia (AD) and von Willebrand's disease (vWD) was first described by Quick in 1967. The clinical course of patients with vWD and AD is characterized by multiple admissions to hospital for gastrointestinal bleeding, necessitating transfusions of packed red cells, factor VIII, and von Willebrand factor concentrates. The therapeutic management of these patients is difficult. Both medical and endoscopic techniques have been tried, but no treatment modality has been completely successful. We describe the clinical course of a patient affected by vWD, who suffered repeated massive gastrointestinal bleeding caused by small bowel AD. Intestinal resection was the only…

Coexistence of Von Willebrand disease and gastrointestinal stromal tumor (G.I.S.T): Case report of a rare and challenge association

2019

Von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by a quantitative (type 1 and 3) or qualitative (type 2) defect of Von Willebrand factor (VWF). Bleeding from the gastrointestinal (GI) tract is not uncommon in VWD and is usually associated with angiodysplasia. We report herein on the management of a patient affected by VWD2B with severe GI bleeding secondary to gastrointestinal stromal tumor (GIST) complicated by deep vein thrombosis (DVT). The current case demonstrated that the hemostatic balance, in RBDs under specific circumstances, can range from a tendency toward a hemorrhagic to normal or prothrombotic state. In these patients, a close collabor…

Gastrointestinal bleeding due to angiodysplasia in patients with type 1 von Willebrand disease: report on association and management

2007

The ISTH bleeding assessment tool as predictor of bleeding events in inherited platelet disorders: Communication from the ISTH SSC Subcommittee on Pl…

2021

Background: The ISTH Bleeding Assessment Tool (ISTH-BAT) has been validated for clinical screening of suspected von Willebrand disease (VWD) and for bleeding prediction. Recently it has been validated for subjects with inherited platelet disorders (IPD) (BAT-VAL study). Objectives: To determine whether the ISTH-BAT bleeding score (BS) predicts subsequent bleeding events requiring treatment in IPD patients. Methods: Patients with IPD, type 1 VWD (VWD-1) and age- and sex-matched healthy controls enrolled in the BAT-VAL study were prospectively followed-up for 2 years and bleeding episodes requiring treatment were recorded. Results: Of the 1098 subjects initially enrolled, 955 were followed-up…

Bleeding Management of Uterine Conization Using a Pasteurized FVIII/VWF Concentrate in a Woman Affected by Type 2A von Willebrand Disease

2007

Dental management in patients with hemostasis alteration

2011

Haemostasis is a mechanism that, through different interdependent biologic processes, has the purpose of ensuring the integrity and permeability of the circulatory system. Hemostasis term means prevention the loss of blood. Interventions or treatments in the oral cavity, in particular those with a possibility of bleeding, represent a risk for patients with disorders of hemostasis. Prevention is the key to avoid bleeding complications after oral surgical procedures and therefore it is essential a detailed medical history of the patient. The appropriate diagnosis and treatment in patients with disorders of hemostasis, depends directly in the understanding of hemostasis standard mechanisms. Em…