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Acquired von Willebrand disease
Shaji Kumar1, Rajiv K Pruthi, William L Nichols
1Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.
Mayo Clinic Proceedings
|February 13, 2002
Summary
Acquired von Willebrand disease (AvWD) is a rare bleeding disorder often seen in older adults. Prompt diagnosis and treatment of underlying conditions are key to managing AvWD symptoms.
Area of Science:
- Hematology
- Internal Medicine
- Pathophysiology
Background:
- Acquired von Willebrand disease (AvWD) is a rare bleeding disorder.
- It predominantly affects elderly patients with no prior bleeding history.
- Mucocutaneous bleeding is the most common presentation.
Purpose of the Study:
- To review the characteristics, pathogenesis, diagnosis, and management of AvWD.
- To highlight the association of AvWD with hematoproliferative and autoimmune disorders.
- To emphasize the importance of recognizing AvWD for timely intervention.
Main Methods:
- Review of existing literature on Acquired von Willebrand disease.
- Analysis of clinical presentations, laboratory findings, and associated conditions.
- Evaluation of current therapeutic strategies and their efficacy.
Main Results:
- AvWD is frequently linked to hematoproliferative disorders like monoclonal gammopathies.
- Pathogenesis involves autoantibodies against von Willebrand factor (vWF) or tumor cell adsorption.
- Laboratory findings typically show prolonged bleeding time and reduced vWF levels.
- Treatment may include desmopressin, factor VIII concentrates, or addressing the underlying illness.
Conclusions:
- AvWD diagnosis requires distinguishing it from congenital forms based on age and history.
- Successful management hinges on treating the underlying associated disorder.
- Increased awareness of AvWD is crucial for effective patient care.