Janet Abrahm, MD, Assistant Professor, Department of Medicine, University of Pennsylvania School of Medicine, and Chief, Hematology/Oncology, Veterans Administration Medical Center, Philadelphia, PA.
The efficacy of intensive plasma exchange in acquired von Willebrand's disease
Article first published online: 5 MAR 2003
Volume 27, Issue 3, pages 234–237, May-June 1987
How to Cite
Silberstein, L.E., Abrahm, J. and Shattil, S.J. (1987), The efficacy of intensive plasma exchange in acquired von Willebrand's disease. Transfusion, 27: 234–237. doi: 10.1046/j.1537-2995.1987.27387235627.x
- Issue published online: 5 MAR 2003
- Article first published online: 5 MAR 2003
- Received November 27, 1985; revised August 1, 1986; accepted August 12, 1986
This study describes the response to therapeutic plasma exchange in a 60-year-old man with Waldenstrom's macroglobulinemia who developed a clinically severe bleeding disorder with laboratory features characteristic of acquired von Willebrand's disease. The patient's plasma levels of factor VIII coagulant activity, von Willebrand's factor antigen, and ristocetin cofactor activity were all less than 15 percent of normal, and the bleeding time was more than 20 minutes. In vitro studies did not demonstrate an inhibitor to factor VIII/von Willebrand factor, nor was a precipitating antibody to von Willebrand's factor antigen found in the patient's plasma. Neither infusions of cryoprecipitate nor combination chemotherapy corrected the clinical or laboratory abnormalities. In contrast, plasma exchange corrected the in vitro coagulation abnormalities and was effective in preventing surgical hemorrhage and controlling severe mucosal bleeding on 11 separate occasions. The current case demonstrates that the clinical and laboratory abnormalities in a patient with acquired von Willebrand's disease can be corrected completely by plasma exchange; it is recommended therefore that plasma exchange be considered as a mode of therapy in symptomatic patients with this disorder.