How I treat patients with inherited bleeding disorders who need anticoagulant therapy

Abstract

Situations that ordinarily necessitate consideration of anticoagulation, such as arterial and venous thrombotic events and prevention of stroke in atrial fibrillation, become challenging in patients with inherited bleeding disorders such as hemophilia A, hemophilia B, and von Willebrand disease. There are no evidence-based guidelines to direct therapy in these patients, and management strategies that incorporate anticoagulation must weigh a treatment that carries a risk of hemorrhage in a patient who is already at heightened risk against the potential consequences of not treating the thrombotic event. In this paper, we review atherothrombotic disease, venous thrombotic disease, and atrial fibrillation in patients with inherited bleeding disorders, and discuss strategies for using anticoagulants in this population using cases to illustrate these considerations.

Arterial and Venous Thrombosis in Haemophilia Patients: Experiences from a Danish Haemophilia Centre

“…5) had multiple minor bleeds on low-dose ASA despite factor replacement. This underlines the need for individual strategies, depending on factor levels and bleeding phenotype, and for a close follow-up and collaboration between the cardiologist and haemophilia centre, as argued by others [12,17]. Only 1 major bleeding event (gastro-intestinal) was recorded, during LMWH treatment.…”

“…Anti-thrombotic treatment in haemophilia is a particular problem. During the last decade, several authors have published their institutional guidelines, expert opinions, and consensus reports on the management of CVD [10,11,12,13,14] and venous thromboembolism (VTE) [12] in PWH, but evidence to back these guidelines is still limited. To add to the general knowledge in this field, we present the experience from our haemophilia centre on the occurrence and management of arterial and venous thrombosis in a population of Danish PWH.…”

“…We found that low-intensity anti-coagulation, as defined by Martin and Key [12], such as single anti-platelet therapy and prophylactic low molecular weight heparin (LMWH), was generally chosen, except during coronary-artery bypass grafting (CABG) where the patients were heparinized due to standard protocols. Acetylsalicylic acid (ASA) was administered in only 4 of 7 coronary events, and none was treated with clopidogrel as suggested in reports recently published by experts.…”

“…Earlier, anti-platelet therapy was rarely used in PWH because of a reluctance to impair primary haemostasis in these patients. However, recent guidelines and expert opinions [10,11,12,13,14,17] advocate that acute coronary disease in PWH should generally be managed as in non-PWH, provided that factor replacement is given, and they consider ASA therapy (with factor levels >5%) and dual anti-platelet therapy (with factor levels >15-30%) to be generally safe. This is supported by 2 recently published case series on 24 PWH with CVD [18,19], who found that revascularization and anti-platelet treatment were generally well-tolerated.…”

“…In our patient, the DVT was managed without anti-coagulant and resolved spontaneously with removal of the risk factors. Martin and Key [12 ]recently put forth their recommendations on DVT treatment in PWH. Their general considerations on whether to treat DVT in PWH include the size and location of the thrombus, the patient's symptoms, and the perceived risk of recurrence (provoking factors).…”