Associated risk factors and arterial occlusions in patients with von Willebrand disease: Analysis of the literature and report of two cases

Abstract

Objective: To investigate the features of all reported cases of arterial occlusion which occurred in patients with Von Willebrand Disease. Patients and methods: Personal records were re-evaluated together with a time-unlimited Pubmed search using pertinent keywords. Occlusive events had to be documented by objective means. All cases of myocardial infarction and other acute coronary syndromes were considered. Cases of angina were instead excluded. Results: 56 patients with arterial occlusion were gathered from the literature (44 cases of acute coronary syndromes and 12 cases of ischemic stroke. Two additional cases were obtained from personal records (1 case of myocardial infarction and 1 case of unstable angina) for a total of 58 patients. Peripheral arterial thrombosis has never been described in von Willebrand disease patients. The majority of these patients presented common risk factors such as smoking, hypertension, diabeties, dyslipidemia, obesity. Replacement therapy, contrary to what seen in hemophilias, had instead a minor role if any. Conclusions: von Willebrand disease is unable to protect from atherosclerosis and atherothrombosis. The common risk factors for arterial disease such as hypertension, diabetiesdyslipidemia and smoking are able to neutralize the potential protective effect of VW Factor deficiency. Despite this, the prevalence of arterial occlusions in von Willebrand disease appears decreased in comparison with the general populations at least in some countries.