Acquired bleeding disorders

Abstract

Acquired bleeding disorders can accompany hematological, neoplastic, autoimmune, cardiovascular or liver diseases, but can sometimes also arise spontaneously. They can manifest as single factor deficiencies or as complex hemostatic abnormalities. This review addresses (a) acquired hemophilia A, an autoimmune disorder characterized by inhibitory autoantibodies against coagulation factor VIII; (b) acquired von Willebrand syndrome in patients with cardiovascular disorders, where shear stress abnormalities result in destruction of von Willebrand factor; and (c) liver function disorders that comprise complex changes in pro‐ and anti‐hemostatic factors, whose clinical implications are often difficult to predict. The article provides an overview on the pathophysiology, diagnostic tests and state‐of‐the‐art treatment strategies.

New onset of acute heavy menstrual bleeding in a 34-year-old woman

“…Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies that inhibit the function of factor VIII. 4,5 The incidence is about 1 to 1.5 cases per million, with a median age of 64 years, affecting men and women equally. 5,7 Acquired hemophilia A is a medical emergency requiring prompt recognition and treatment, especially in a bleeding patient.…”

“…4,5 The incidence is about 1 to 1.5 cases per million, with a median age of 64 years, affecting men and women equally. 5,7 Acquired hemophilia A is a medical emergency requiring prompt recognition and treatment, especially in a bleeding patient. Its hallmark presentation consists of moderate-tosevere bleeding (large hematomas, or mucocutaneous or soft tissue bleeds), extensive bruising or bleeding from unusual sites, without a personal or family history of bleeding.…”

“…Acquired hemophilia A is confirmed by a low factor VIII level and the presence of a factor VIII inhibitor, which can be measured in specialized coagulation laboratories. 4,5 For completeness, we also tested von Willebrand factor antigen and activity levels, which were within normal range, ruling out acquired von Willebrand syndrome. Based on our investigations, we diagnosed acquired hemophilia A in our patient.…”

“…Immunosuppression with prednisone at 1 mg/kg daily was started as an initial treatment to eradicate the inhibitor. Given that 30%-50% of patients with acquired hemophilia A will have an underlying malignancy or autoimmune condition, 5 additional investigations were completed, including antinuclear antibodies, rheumatoid factor, protein electrophoresis, and imaging of her chest, abdomen and pelvis. Her work-up revealed an immunoglobulin G κ monoclonal protein level of 25 g/L.…”

Type 2A von Willebrand disease and systemic sclerosis: Vonicog alfa reduced gastrointestinal bleeding

“…Primary and secondary (acquired) forms of VWD exist 1 . The latter can be associated with various underlying conditions, such as autoimmune diseases, where VWF function is impaired by binding to autoantibodies 2 . Apart from its role in primary hemostasis, VWF participates in the regulation of angiogenesis.…”