Presentation Details
| Bleeding Duet: A case of simultaneous acquired factor VIII deficiency and acquired von Willebrand syndrome Richard Yu1, Mathew Strelau1, Thunisa Shanmugalingham1 , Mackenzie Bowman2, Arnaud Bonnefoy3, Paula James2, Chai Phua1. 1University of Western Ontario, Department of Hematology, London, ON, Canada.2Queens University, Department of Hematology, Kingston, ON, Canada.3University of Montreal, Department of Hematology, Montreal, QC, Canada |
Abstract
Background: Acquired factor VIII (aFVIII) deficiency and acquired von Willebrand syndrome (AVWS) are both rare bleeding disorders and have only been reported in isolation. aFVIII is typically associated with lymphoproliferative disorders or autoimmune conditions that predisposes to autoantibody formation. The etiology of AVWS is much more heterogenous. They may also be associated with autoantibodies. However, other mechanisms include mechanical stress mediated proteolysis and adsorption from circulation mediated by glycoprotein Ib have been described as well. Here we present a case of concurrent aFVIII deficiency and AVWS. Case presentation: 73-year-old lady with history of prior stroke, STEMI, collagenous colitis, and no recent anticoagulation history, presented with chest pain and gastrointestinal bleeding. Initial bloodwork revealed hemoglobin of 49g/L and found to have an isolated elevated PTT at 64s. The workup of her PTT identified undetectable factor VIII levels <0.01 U/mL and factor VIII inhibitor was elevated at 28.8 Bethesda Units. Furthermore, von Willebrand Antigen was low at 0.12U/mL and activity was low at <0.15U/mL. Subsequent ELISA was performed to confirm presence of a distinct von Willebrand inhibitor. The patient was started on immunosuppression with oral prednisone, IV cyclophosphamide and IVIG. She also received factor VIII replacement, Obizur. Her autoantibody levels decreased in response to this treatment and her clinical bleeding has stopped. Further workup was negative for rheumatologic conditions, myeloma and solid organ malignancy. Colonoscopy revealed multiple angiodysplasias that were treated with argon plasma coagulation and echocardiogram was negative for aortic stenosis. The patient was successfully discharged in remission with recovery of her factor VIII, von Willebrand antigen and activity levels and followed up as an outpatient in the bleeding disorders clinic. Conclusion: To the authors knowledge, this is the first documented instance of two acquired bleeding disorders that occurred simultaneously in a single individual. Here we present the diagnostic steps taken to identify and then prove the presence of two distinct acquired autoimmune mediated bleeding entities and documented positive longitudinal response to immunosuppression.
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No part of this publication may be reproduced, distributed, or transmitted in any form or by any means, including photocopying, recording, or other electronic or mechanical methods, without the prior written permission of the author.