Abstract
IntroductionAcquired factor VIII deficiency is a rare entity that can lead to severe and life-threatening bleeding. We describe a case of severe bleeding from the tongue secondary to acquired hemophilia and discuss treatment options, including aminocaproic acid and recombinant factor VIII, which have not been widely reported in the literature for the management of such patients.Case presentationA 94-year-old Caucasian man presented to our institution with diffuse bruising and extensive bleeding from the tongue secondary to mechanical trauma. He had no prior history of bleeding and his medical history was unremarkable except for dementia and hypertension. Coagulation studies revealed a prolonged activated partial thromboplastin time and a mixing study was consistent with the presence of an inhibitor. Quantitative assays revealed a reduced level of factor VIII activity (1%) and the presence of a factor VIII inhibitor, measured at seven Bethesda units, in the serum. Oral prednisone therapy (60mg/day) was given. He also received intravenous aminocaproic acid and human concentrate of factor VIII (Humate-P) and topical anti-thrombolytic agents (100 units of topical thrombin cream). His hospital course was prolonged because of persistent bleeding and the development of profuse melena. He required eight units of packed red blood cells for transfusion. Hospitalization was also complicated by bradycardia of unclear etiology, which started after infusion of aminocaproic acid. His activated partial thromboplastin time gradually normalized. He was discharged to a rehabilitation facility three weeks later with improving symptoms, stable hematocrit and resolving bruises.ConclusionsClinicians should suspect a diagnosis of acquired hemophilia in older patients with unexplained persistent and profound bleeding from uncommon soft tissues, including the tongue. Use of factor VIII (Humate-P) and aminocaproic acid can be useful in this coagulopathy but clinicians should be aware of possible life-threatening side effects in older patients, including bradycardia.
Highlights
Acquired factor VIII deficiency is a rare entity that can lead to severe and life-threatening bleeding
The presence of an inhibitor of coagulation was diagnosed via prolonged activated partial thromboplastin time and a mixing study that did not correct with the addition of normal plasma (partial thromboplastin time (PTT) 36.4 seconds when an immediate mixing test was performed, with a ratio of patient’s plasma to normal plasma of 1:1) (Figure 1)
Quantitative assays revealed a reduced level of factor VIII activity (1%) and the presence of factor VIII inhibitor measured at 7 Bethesda units (BU) in the serum
Summary
Acquired hemophilia A is an extremely rare clinical entity. Use of Humate-P can be useful in this coagulopathy, whereas use of aminocaproic acid in states of acquired hemophilia may sometimes be associated with life-threatening complications including bradycardia. Consent Written informed consent was obtained from the patient’s of kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Authors’ contributions TK analyzed and interpreted the patient data and was a major contributor in writing the manuscript. JR analyzed the patient data and contributed in writing the manuscript. RP and BE analyzed and interpreted the patient data and were major contributors in writing the manuscript. All authors read and approved the final manuscript. Competing interests The authors declare that they have no competing interests
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