Abstract
Development of autoantibodies against coagulation factors is an uncommon bleeding disorder associated with cancer, autoimmune conditions, pregnancy, or no apparent disease. Spontaneous FVIII inhibitors are the most frequently encountered; those against FXI have been only anecdotally reported. We report a case of acquired FXI inhibitor presenting as fatal intracranial spontaneous bleeding in an elderly patient with history of cancer and previous transfusions. Few cases of acquired FXI inhibitor have been reported in association with connective tissue disease, cancer, or surgery. Bleeding includes mucocutaneous bleeding, postsurgical hemorrhage, or life-threatening events. Treatment consists of arresting the bleeding and inhibitor eradication. High degree of suspicion is essential to promptly diagnose and treat this uncommon condition.
Highlights
Immune-mediated development of autoantibodies against coagulation factors in patients without congenital deficiency is a rare but potentially life-threatening bleeding disorder [1]
Acquired hemophilia A (AHA) has been associated with malignancy, autoimmune disorders, pregnancy, multiple transfusions, or no apparent disease [3]
A 90-year-old man presented with decreased level of consciousness and generalised tonic-clonic seizure
Summary
Immune-mediated development of autoantibodies against coagulation factors in patients without congenital deficiency is a rare but potentially life-threatening bleeding disorder [1]. Acquired hemophilia A (AHA) is the most common form of the disorder and is caused by inhibitory antibodies that neutralize coagulation factor VIII (FVIII) activity [2]. Inhibitors against other clotting factors are much rarer [1]; in particular those against FXI have been only anecdotally reported [4,5,6,7,8,9,10,11,12]. We report a case of acquired FXI inhibitors presenting as spontaneous intracranial bleeding in an elderly patient with history of cancer and briefly review current literature on clinical characteristics and management strategies of this uncommon condition
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