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[Acquired von Willebrand syndrome]
D W de Lange1, R Fijnheer, S Wittebol
1Universitair Medisch Centrum, afd. Interne Geneeskunde, F02.107, Heidelberglaan 100, 3508 GA Utrecht. d.w.delange@azu.nl
Nederlands Tijdschrift Voor Geneeskunde
|October 7, 2003
Summary
Acquired Von Willebrand syndrome (AVWS) in two patients resulted from monoclonal gammopathy. Treatment involved immunoglobulin and factor concentrates, leading to successful surgical outcomes and management of bleeding tendencies.
Area of Science:
- Hematology
- Internal Medicine
Background:
- Acquired Von Willebrand syndrome (AVWS) is a rare bleeding disorder associated with various underlying conditions, including paraproteinemia and lymphoproliferative diseases.
- Prevalence of AVWS is estimated between 0.04% and 0.13%.
Observation:
- Two cases of AVWS are presented: a 69-year-old male with epistaxis and a 64-year-old female with hemorrhagic diathesis.
- Both patients had monoclonal gammopathy as the underlying cause of AVWS.
- The male patient underwent hip surgery successfully after treatment with immunoglobulin; the female patient was advised on prophylactic treatment for invasive procedures.
Findings:
- Diagnosis of AVWS relies on clinical history and laboratory tests including VWF antigen, VWF propeptide, activated partial thromboplastin time, and factor VIII.
- Identifying and treating the underlying disease is crucial for managing AVWS.
- Symptomatic treatment options include desmopressin, VWF concentrates, and intravenous immunoglobulin.
Implications:
- Effective management of AVWS involves a dual approach: symptomatic treatment of bleeding and addressing the root cause.
- Treatment of the underlying condition can potentially resolve AVWS.
- Early recognition and diagnosis are vital for preventing complications in patients with AVWS.