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New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome

Acquired hemophilia A and acquired von Willebrand syndrome are rare, but life-threatening bleeding disorders that require prompt diagnosis and treatment by hematologists. Acquired hemophilia A is defined as an acquired severe bleeding tendency caused by autoantibody formation against coagulation fac...

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Autor principal: Leebeek, Frank W. G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8171371/
https://www.ncbi.nlm.nih.gov/pubmed/34095769
http://dx.doi.org/10.1097/HS9.0000000000000586
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author Leebeek, Frank W. G.
author_facet Leebeek, Frank W. G.
author_sort Leebeek, Frank W. G.
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description Acquired hemophilia A and acquired von Willebrand syndrome are rare, but life-threatening bleeding disorders that require prompt diagnosis and treatment by hematologists. Acquired hemophilia A is defined as an acquired severe bleeding tendency caused by autoantibody formation against coagulation factor VIII. Acquired von Willebrand syndrome is characterized by a new onset bleeding tendency caused by a reduced concentration and/or function of von Willebrand factor. These disorders are associated with a variety of underlying disorders, including various hematological malignancies, for example, plasma cell disorders, lymphoproliferative disorders, monoclonal gammopathy of undetermined significance, and myeloproliferative neoplasms. It is of utmost important to recognize these acquired bleeding disorders in these patients who are at risk for severe bleeding, and to perform additional diagnostic hemostasis laboratory evaluation. This will enable immediate diagnosis of the acquired bleeding disorder and management of both the bleeding episodes and the causative underlying disorder. In recent years, several new etiological factors for acquired hemophilia A, such as treatment with immune checkpoint inhibitors or DPP-4 inhibitors and SARS-CoV2 infection, and for acquired von Willebrand syndrome, for example, left ventricular assist devices, have been identified and also new treatment options have become available. In this concise review, the most recent data on etiology, diagnosis, and treatment of acquired bleeding disorders are presented and discussed.
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spelling pubmed-81713712021-06-03 New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome Leebeek, Frank W. G. Hemasphere Review Acquired hemophilia A and acquired von Willebrand syndrome are rare, but life-threatening bleeding disorders that require prompt diagnosis and treatment by hematologists. Acquired hemophilia A is defined as an acquired severe bleeding tendency caused by autoantibody formation against coagulation factor VIII. Acquired von Willebrand syndrome is characterized by a new onset bleeding tendency caused by a reduced concentration and/or function of von Willebrand factor. These disorders are associated with a variety of underlying disorders, including various hematological malignancies, for example, plasma cell disorders, lymphoproliferative disorders, monoclonal gammopathy of undetermined significance, and myeloproliferative neoplasms. It is of utmost important to recognize these acquired bleeding disorders in these patients who are at risk for severe bleeding, and to perform additional diagnostic hemostasis laboratory evaluation. This will enable immediate diagnosis of the acquired bleeding disorder and management of both the bleeding episodes and the causative underlying disorder. In recent years, several new etiological factors for acquired hemophilia A, such as treatment with immune checkpoint inhibitors or DPP-4 inhibitors and SARS-CoV2 infection, and for acquired von Willebrand syndrome, for example, left ventricular assist devices, have been identified and also new treatment options have become available. In this concise review, the most recent data on etiology, diagnosis, and treatment of acquired bleeding disorders are presented and discussed. Lippincott Williams & Wilkins 2021-06-01 /pmc/articles/PMC8171371/ /pubmed/34095769 http://dx.doi.org/10.1097/HS9.0000000000000586 Text en Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review
Leebeek, Frank W. G.
New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome
title New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome
title_full New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome
title_fullStr New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome
title_full_unstemmed New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome
title_short New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome
title_sort new developments in diagnosis and management of acquired hemophilia and acquired von willebrand syndrome
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8171371/
https://www.ncbi.nlm.nih.gov/pubmed/34095769
http://dx.doi.org/10.1097/HS9.0000000000000586
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