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Massive postoperative intramuscular bleeding in acquired von Willebrand's disease
M Krebs1, B Meyer, P Quehenberger
1Department of Medicine III, University of Vienna Medical School, Austria.
Abstract:
We describe a case of acquired von Willebrand's disease (vWD) associated with monoclonal gammopathy with undetermined significance (MGUS) in a 54-year-old man who was admitted with hemarthrosis and extensive thigh muscle hematoma following arthroscopic surgery and postoperative prophylaxis with low molecular weight heparin. Coagulation tests were compatible with acquired vWD: prolonged activated partial thromboplastin time (aPTT) (56.1 s), decreased levels of factor VIII coagulant activity (23%), low concentrations of von Willebrand's factor (vWF) antigen (13%), and undetectable ristocetin cofactor activity (<10%). Infusion of a vWF-containing factor VIII concentrate failed to normalize the plasma levels of vWF-related parameters. Only additional intravenous administration of immunoglobulins led to a transient normalization of ristocetin cofactor activity, vWF antigen, and factor VIII coagulant activity. While the spontaneous bleeding tendency in this case was mild, surgery and administration of prophylactic doses of low molecular weight heparin led to life-threatening bleeding.
Insights
This case study details acquired von Willebrand
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Acquired von Willebrand's disease (vWD) is a rare bleeding disorder.
- Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant condition.
- The association between acquired vWD and MGUS is infrequently reported.
Observation:
- A 54-year-old male presented with hemarthrosis and hematoma post-arthroscopic surgery.
- Postoperative low molecular weight heparin prophylaxis exacerbated bleeding.
- Coagulation tests revealed prolonged aPTT, decreased Factor VIII, and low von Willebrand factor (vWF) levels.
Findings:
- The patient exhibited laboratory findings consistent with acquired vWD.
- vWF-containing factor VIII concentrate infusion was ineffective.
- Intravenous immunoglobulin therapy resulted in transient normalization of vWF parameters.
Implications:
- This case highlights a rare association between MGUS and acquired vWD.
- It underscores the potential for severe bleeding complications with anticoagulation in such patients.
- Intravenous immunoglobulin may be a therapeutic option for managing acquired vWD in MGUS.