Thrombotic complications in adult patients with severe single coagulation factor or platelet defects - an overview.

INTRODUCTION: Even though thrombotic events are rare in patients with coagulation deficiencies, several cases of both arterial and venous thromboses have been reported in patients with single coagulation factor defects and platelet defects. Thromboses have been described both in hemophilia A and B, von Willebrand disease as well as in many other rare congenital coagulation factor and platelet defects. Thromboses may also occur in patients with acquired hemophilia and in patients with severe thrombocytopenia due to hematological malignancies or intensive chemotherapy. Areas covered: We searched in the PubMed database to identify scientific publications describing patients with bleeding disorders and thromboses and published suggestions/guidelines for the treatment of thromboses in such patients. Expert commentary: The article gives a review of published case reports/studies of thromboses in these patients. Thromboses are rare in all these diseases. There is generally a lack controlled clinical studies for the use of anticoagulation therapy in such patients, and the article gives an overview of published suggestions and expert opinions based on the experience at large centers for treatment of thromboses in patients with congenital coagulation defects, acquired hemophilia and severe thrombocytopenia.