Karan L. Chohan, Rajiv K. Pruthi, Saurabh Zanwar, Jonas Paludo, Ronald Go, Animesh Pardanani, Aneel Ashrani, Joselle M. Cook, Carrie A. Thompson, Asher Chanan-Khan, Sikander Ailawadhi, Thomas M. Habermann, Thomas E. Witzig, Morie A. Gertz, David Dingli, Fransis K. Buadi, Angela Dispenzieri, Nelson Leung, Shaji K. Kumar, Vincent Rajkumar, William L. Nichols, Robert A. Kyle, Stephen M. Ansell, Prashant Kapoor, Meera Sridharan, Jithma P. Abeykoon
{"title":"继发于瓦尔登斯特伦巨球蛋白血症的获得性冯-威廉综合征的临床影响:未被充分认识的大出血事件源头","authors":"Karan L. Chohan, Rajiv K. Pruthi, Saurabh Zanwar, Jonas Paludo, Ronald Go, Animesh Pardanani, Aneel Ashrani, Joselle M. Cook, Carrie A. Thompson, Asher Chanan-Khan, Sikander Ailawadhi, Thomas M. Habermann, Thomas E. Witzig, Morie A. Gertz, David Dingli, Fransis K. Buadi, Angela Dispenzieri, Nelson Leung, Shaji K. Kumar, Vincent Rajkumar, William L. Nichols, Robert A. Kyle, Stephen M. Ansell, Prashant Kapoor, Meera Sridharan, Jithma P. Abeykoon","doi":"10.1038/s41375-024-02393-6","DOIUrl":null,"url":null,"abstract":"<p>An under-recognized source of bleeding in Waldenström macroglobulinemia (WM) is the development of an acquired von Willebrand syndrome (AVWS) [1]. In WM, the von Willebrand Factor (vWF) glycoprotein can be degraded due to autoantibody destruction, increased shear stress due to hyperviscosity, or sequestered due to adsorption onto malignant cells, leading to the development of AVWS (AVWS-WM) [1, 2]. However, there is currently a paucity of published literature characterizing the clinical features associated with AVWS-WM and, specifically, the change in bleeding-related symptoms through treatment [3,4,5,6]. In this study, we assessed the prevalence of AVWS-WM in patients with WM treated in a tertiary care center and evaluated the associated clinical manifestations and outcomes of patients with AVWS-WM compared to those with WM and without AVWS.</p><p>This retrospective cohort study evaluated patients with active WM seen at Mayo Clinic and affiliated practices from January 2002 to January 2022. Patients were identified using the Mayo Clinic Data Explorer, and those who underwent vWF testing and had confirmed a diagnoses of WM and AVWS were included [7]. A matched control cohort (patients with WM and without AVWS) with a 5:1 ratio based on age, sex, and diagnosis date was also established. Detailed methodologies are available in the Supplementary Materials.</p>","PeriodicalId":18109,"journal":{"name":"Leukemia","volume":null,"pages":null},"PeriodicalIF":12.8000,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"The clinical impact of acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia: an underrecognized source of major bleeding events\",\"authors\":\"Karan L. Chohan, Rajiv K. Pruthi, Saurabh Zanwar, Jonas Paludo, Ronald Go, Animesh Pardanani, Aneel Ashrani, Joselle M. Cook, Carrie A. Thompson, Asher Chanan-Khan, Sikander Ailawadhi, Thomas M. Habermann, Thomas E. Witzig, Morie A. Gertz, David Dingli, Fransis K. Buadi, Angela Dispenzieri, Nelson Leung, Shaji K. Kumar, Vincent Rajkumar, William L. Nichols, Robert A. Kyle, Stephen M. Ansell, Prashant Kapoor, Meera Sridharan, Jithma P. 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The clinical impact of acquired von Willebrand syndrome secondary to Waldenström macroglobulinemia: an underrecognized source of major bleeding events
An under-recognized source of bleeding in Waldenström macroglobulinemia (WM) is the development of an acquired von Willebrand syndrome (AVWS) [1]. In WM, the von Willebrand Factor (vWF) glycoprotein can be degraded due to autoantibody destruction, increased shear stress due to hyperviscosity, or sequestered due to adsorption onto malignant cells, leading to the development of AVWS (AVWS-WM) [1, 2]. However, there is currently a paucity of published literature characterizing the clinical features associated with AVWS-WM and, specifically, the change in bleeding-related symptoms through treatment [3,4,5,6]. In this study, we assessed the prevalence of AVWS-WM in patients with WM treated in a tertiary care center and evaluated the associated clinical manifestations and outcomes of patients with AVWS-WM compared to those with WM and without AVWS.
This retrospective cohort study evaluated patients with active WM seen at Mayo Clinic and affiliated practices from January 2002 to January 2022. Patients were identified using the Mayo Clinic Data Explorer, and those who underwent vWF testing and had confirmed a diagnoses of WM and AVWS were included [7]. A matched control cohort (patients with WM and without AVWS) with a 5:1 ratio based on age, sex, and diagnosis date was also established. Detailed methodologies are available in the Supplementary Materials.
期刊介绍:
Title: Leukemia
Journal Overview:
Publishes high-quality, peer-reviewed research
Covers all aspects of research and treatment of leukemia and allied diseases
Includes studies of normal hemopoiesis due to comparative relevance
Topics of Interest:
Oncogenes
Growth factors
Stem cells
Leukemia genomics
Cell cycle
Signal transduction
Molecular targets for therapy
And more
Content Types:
Original research articles
Reviews
Letters
Correspondence
Comments elaborating on significant advances and covering topical issues