Abstract Number: PB0945
Meeting: ISTH 2021 Congress
Background: Type 2B Von Willebrand Disease (VWD) is an inherited bleeding disorder caused by changes in Von Willebrand Factor (VWF) that enhances binding of VWF to GPIb on platelets. Patients with this subtype have a more severe bleeding phenotype compared with other type 2.
Aims: To describe the challenge of managing a patient with VWD and multiple thromboembolic risk factors.
Methods: We report the case of a 74-year-old male with type 2B VWD (variant found in exon 28), responsible for thrombocytopenia and high bleeding risk. He presented high thrombotic risk due to atrial fibrillation, arterial hypertension, critical left renal artery stenosis, parietal thrombosis in the left common iliac artery, as well as positive for anticardiolipin antibodies. Patient data was collected from medical records.
Results: After an evaluation by a multidisciplinary team, percutaneous closure of the left atrial appendage was carried out in order to avoid anticoagulation therapy and started treatment with antiplatelet agent. During the past years, major and frequent gastrointestinal bleeding episodes occurred; one of them was life-threatening. After discussing the risk and benefits of different treatment options for the condition presented, prophylaxis with VWF/FVIII concentrated (1:1 balance of VWF and FVIII) was started: 1500 IU/twice a week to maintain FVIII:C concentrations under 150 IU/dL in order to reduce the thromboembolic risk. Therapy with VWF/FVIII concentrate significantly reduced the frequency and severity of bleeding episodes with stabilized hemoglobin levels, and bleeding risk was reassessed throughout the course of treatment. Thrombotic episodes or other side effects were not observed following the infusion of the treatment. To reduce the thromboembolic risk we valued the benefits of switching to high-purity VWF concentrate.
Conclusions: This case illustrates the challenging nature of VWD and multiple prothrombotic risk factors. Prophylaxis with VWF/FVIII showed a reduction in transfusion dependence and significant improvement in quality of life.
To cite this abstract in AMA style:Merchan Muñoz B, Herrero Martín S, Nuevo López MI, Mora Argumánez M. Finding the Balance between Bleeding Risk and Thromboembolic Risk: A Case Report [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/finding-the-balance-between-bleeding-risk-and-thromboembolic-risk-a-case-report/. Accessed November 28, 2021.
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