Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma

Acquired von Willebrand syndrome (AVWS) is an increasingly recognized entity with numerous potential underlying etiologies. Most commonly implicated are lymphoproliferative, myeloproliferative, cardiovascular, and autoimmune disorders. Unlike inherited von Willebrand disease (vWD), AVWS tends to pre...

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Main Authors: Zachary Wolfe, Bradley Lash
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2017/9862620
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author Zachary Wolfe
Bradley Lash
author_facet Zachary Wolfe
Bradley Lash
author_sort Zachary Wolfe
collection DOAJ
description Acquired von Willebrand syndrome (AVWS) is an increasingly recognized entity with numerous potential underlying etiologies. Most commonly implicated are lymphoproliferative, myeloproliferative, cardiovascular, and autoimmune disorders. Unlike inherited von Willebrand disease (vWD), AVWS tends to present at an older age and without a family history of vWD. Treatment is directed at the underlying etiology if one is uncovered, as well as treatment and prevention of bleeding. Here, we present a rare case of AVWS secondary to Waldenström macroglobulinemia which went unrecognized for several years but resolved promptly with treatment. The potential mechanisms of AVWS secondary to monoclonal gammopathies are discussed as well as strategies to treat and prevent bleeding in these patients.
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institution Kabale University
issn 2090-6560
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series Case Reports in Hematology
spelling doaj-art-3966cedb009b4bfeb5ad0270c3fd07762025-02-03T01:01:37ZengWileyCase Reports in Hematology2090-65602090-65792017-01-01201710.1155/2017/98626209862620Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic LymphomaZachary Wolfe0Bradley Lash1Department of Internal Medicine, Guthrie Clinic/Robert Packer Hospital, Sayre, PA, USADepartment of Hematology and Oncology, Guthrie Clinic/Robert Packer Hospital, Sayre, PA, USAAcquired von Willebrand syndrome (AVWS) is an increasingly recognized entity with numerous potential underlying etiologies. Most commonly implicated are lymphoproliferative, myeloproliferative, cardiovascular, and autoimmune disorders. Unlike inherited von Willebrand disease (vWD), AVWS tends to present at an older age and without a family history of vWD. Treatment is directed at the underlying etiology if one is uncovered, as well as treatment and prevention of bleeding. Here, we present a rare case of AVWS secondary to Waldenström macroglobulinemia which went unrecognized for several years but resolved promptly with treatment. The potential mechanisms of AVWS secondary to monoclonal gammopathies are discussed as well as strategies to treat and prevent bleeding in these patients.http://dx.doi.org/10.1155/2017/9862620
spellingShingle Zachary Wolfe
Bradley Lash
Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma
Case Reports in Hematology
title Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma
title_full Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma
title_fullStr Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma
title_full_unstemmed Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma
title_short Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma
title_sort acquired von willebrand syndrome in igm monoclonal gammopathy as the presentation of lymphoplasmacytic lymphoma
url http://dx.doi.org/10.1155/2017/9862620
work_keys_str_mv AT zacharywolfe acquiredvonwillebrandsyndromeinigmmonoclonalgammopathyasthepresentationoflymphoplasmacyticlymphoma
AT bradleylash acquiredvonwillebrandsyndromeinigmmonoclonalgammopathyasthepresentationoflymphoplasmacyticlymphoma