Acquired von Willebrand's syndrome: A single institution experience
Article first published online: 21 MAR 2003
Copyright © 2003 Wiley-Liss, Inc.
American Journal of Hematology
Volume 72, Issue 4, pages 243–247, April 2003
How to Cite
Kumar, S., Pruthi, R. K. and Nichols, W. L. (2003), Acquired von Willebrand's syndrome: A single institution experience. Am. J. Hematol., 72: 243–247. doi: 10.1002/ajh.10298
- Issue published online: 21 MAR 2003
- Article first published online: 21 MAR 2003
- Manuscript Accepted: 15 DEC 2002
- Manuscript Received: 15 AUG 2002
- acquired von Willebrand's syndrome;
- von Willebrand factor;
- monoclonal gammopathy
Acquired von Willebrand's disease or syndrome (AVWS) is a rare bleeding disorder distinguished from congenital von Willebrand's disease by age at presentation and absence of personal and family history of bleeding disorders. We report on 22 patients with AVWS seen over 25 years. Mean age at diagnosis was 61.3 years (range 38–86 years); most patients had a spontaneous or a post-operative hemorrhage at presentation. Gastrointestinal bleeding and epistaxis were the most common spontaneous symptoms. Bleeding time was prolonged in most patients, associated with marked reductions in plasma von Willebrand factor antigen and ristocetin cofactor activity. Plasma VWF multimer distribution was normal (type 1 pattern) in 5 patients, indeterminate (no multimers detectable) in 6 patients (type 3 pattern), and abnormal (decreased higher-molecular-weight multimers, type 2 pattern) in 11 patients. None of 17 patients tested had an inhibitor of ristocetin cofactor activity. An underlying malignant or benign hematologic disease was found in 18 patients, and 1 patient had Crohn's disease. Desmopressin was effective in only half the patients so treated, but all patients responded to treatment with VWF-containing concentrates. Resolution of AVWS occurred with therapy of lymphoma (1 patient) and chronic lymphocytic leukemia (1 patient). Sixteen patients were alive at last follow-up; no deaths were related to bleeding. AVWS may be more prevalent than has been appreciated; we estimate up to 0.04%. Awareness of the existence of AVWS is essential for diagnosis and appropriate management. Therapy of associated diseases may improve the bleeding disorder. Am. J. Hematol. 72:243–247, 2003. © 2003 Wiley-Liss, Inc.