## Abstract Acquired von Willebrand disease (aVWD) is a rare bleeding disorder that mimics congenital VWD in previously healthy individuals; it is most frequently associated with monoclonal gammopathy. Hemostatic therapy of aVWD is challenging due to the extremely shortened half‐life of endogenous
Retrospective review of the management of elective surgery with desmopressin and clotting factor concentrates in patients with von Willebrand disease
✍ Scribed by Ioana C. Nitu-Whalley; Anja Griffioen; Chris Harrington; Christine A. Lee
- Publisher
- John Wiley and Sons
- Year
- 2001
- Tongue
- English
- Weight
- 32 KB
- Volume
- 66
- Category
- Article
- ISSN
- 0361-8609
- DOI
- 10.1002/ajh.1058
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✦ Synopsis
Abstract
Limited data are available regarding optimal treatment with desmopressin (DDAVP) or intermediate‐purity FVIII concentrates rich in VWF (CFCs) in patients with von Willebrand disease (VWD) who undergo planned surgery. We undertook a retrospective review over 10 years (1988–1997) and identified 27 patients treated with DDAVP for 35 surgical events and 38 patients who received CFCs for 68 elective surgical events. Tranexamic acid was usually added for mucosal surgery. The FVIII:C levels and the severity of surgery were used to determine the frequency and the doses of postoperative treatment. For major surgery the median pre‐ and post‐operative doses of CFCs were 54 and 43 IU/kg, respectively, and for minor surgery the median doses varied between 34 and 52 IU/kg preoperatively and between 23 and 37 IU/kg postoperatively. The effectiveness of haemostasis was excellent in 32 events (91%) treated with DDAVP and in 56 events (82%) treated with CFCs. It is concluded that patients with VWD do not carry an increased operative risk if appropriate therapy is given. Am. J. Hematol. 66:280–284, 2001. © 2001 Wiley‐Liss, Inc.
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## Abstract Decreased von Willebrand factor (VWF)‐cleaving protease activity (<5%) has been implicated in patients with congenital thrombotic thrombocytopenic purpura–hemolytic uremic syndrome (Upshaw‐Schulman syndrome) and associated with mutations within the __ADAMTS13__ gene. In this report, we