ArticleResearch and practice in thrombosis and haemostasis2021
Acquired von Willebrand syndrome in monoclonal gammopathy - A scoping review on hemostatic management.
Article in Research and practice in thrombosis and haemostasis, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 34 citations in OpenAlex.
- Diagnostic and therapeutic challenges in rare hematologic entities: monoclonal gammopathy of thrombotic and bleeding significance.Haematologica · 2026Review
- von Willebrand disease: an illustrated review.Research and practice in thrombosis and haemostasis · 2026Review
- Monoclonal gammopathy of clinical significance: a multisystem review.Blood cancer journal · 2026Review
- Optimizing treatment for Waldenström macroglobulinemia-associated acquired von Willebrand syndrome: a case report and literature review.Frontiers in oncology · 2026Article
- Perioperative management of transcatheter aortic valve implantation in acquired von Willebrand syndrome secondary to monoclonal gammopathy: a case report.European heart journal. Case reports · 2025Article
- Acquired von Willebrand syndrome secondary to monoclonal gammopathy: a single-center case series.Therapeutic advances in hematology · 2025Article
- Article
- Percutaneous Left Atrial Appendage Closure in a Patient With Acquired Von Willebrand Disease and Atrial Fibrillation.Cureus · 2024Article
- Acquired von Willebrand syndrome in a patient with monoclonal gammopathy of unknown significance: A case report.Clinical case reports · 2024Article
- Surprise diagnosis of acquired von Willebrand syndrome in a patient previously thought to have type III von Willebrand disease: evaluation and periprocedural management.BMJ case reports · 2023Article
- Anticoagulation and antiplatelet therapy in hereditary hemorrhagic telangiectasia: A scoping review.Thrombosis research · 2023Article
- Acquired von Willebrand Syndrome in a Patient with Multiple Comorbidities, Including MALT Lymphoma with IgA Monoclonal Gammopathy and Hyperviscosity Syndrome.Internal medicine (Tokyo, Japan) · 2023Article
- Successful use of lenalidomide to treat refractory acquired von Willebrand disease associated with monoclonal gammopathy.Annals of hematology · 2022Article
- Bleeding Propensity in Waldenström Macroglobulinemia: Potential Causes and Evaluation.Thrombosis and haemostasis · 2022Review
- Successful treatment of acquired von Willebrand syndrome associated with monoclonal gammopathy : Breaking a dangerous bond.Wiener klinische Wochenschrift · 2022Article
- Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment.Frontiers in immunology · 2022Review
- How to manage bleeding disorders in aging patients needing surgery.Hematology. American Society of Hematology. Education Program · 2021Article
- Continuous-infusion von Willebrand factor concentrate is effective for the management of acquired von Willebrand disease.Blood advances · 2021Article
- Acquired von Willebrand syndrome in monoclonal gammopathy - A scoping review on hemostatic management.Research and practice in thrombosis and haemostasis · 2021Article
- Clinical Spectrum, Subtype Distribution, and Treatment Outcomes in von Willebrand Disease: A Prospective Study from a Hemophilia Treatment Center in Pakistan.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisObservational
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
backgroundAcquired von Willebrand syndrome (AVWS) has been associated with monoclonal gammopathy of undetermined significance (MGUS), with limited data on its management.
methodsWe conducted a systematic literature search in Medline (Ovid), Embase, and Scopus up to September 11, 2019, for studies reporting on the management of AVWS associated with MGUS (AVWS-MGUS). Data on patient characteristics, laboratory parameters at presentation, and clinical and laboratory outcomes were extracted.
objectivesTo describe the clinical presentation and outcomes of different therapeutic approaches.
resultsSeventy-five studies were included in the final review, for a total of 137 patients. Most patients had von Willebrand factor ristocetin cofactor activity <30 IU/dL (86.6%) and factor VIII levels <50 IU/dL (91.8%). Bleeding severity ranged from no bleeding (16.1%) to minor bleeding (46.4%) and major bleeding (37.5%). The overall clinical success rates for 1-deamino-8-D-arginine vasopressin (DDAVP), factor replacement therapy, and intravenous immunoglobulin (IVIG) were 43.8%, 33.3%, and 85.4%, respectively. The laboratory response rates for DDAVP, factor replacement therapy, and IVIG were 39.0%, 62.9%, and 88.6%, respectively. Several other treatments were also reported in small numbers, out of which myeloma-directed therapies, plasma exchange, recombinant factor VIIa, and antifibrinolytics appeared most successful, while immunosuppressive agents were largely ineffective.
conclusionIVIG appears to be an effective treatment for AVWS-MGUS bleeding, conferring a high clinical success rate with measurable laboratory outcomes; albeit temporary. DDAVP and factor replacement therapy may be partially successful in controlling minor bleeds, but not major bleeds. Other less commonly used agents may be effective in certain cases, although data are limited.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.