Retrospective review of the management of elective surgery with desmopressin and clotting factor concentrates in patients with von Willebrand disease

Limited data are available regarding optimal treatment with desmopressin (D DAVP) or intermediate-purity FVIII concentrates rich in VWF (CFCs) in patie nts with von Willebrand disease (VWD) who undergo planned surgery. We under took a retrospective review over 10 years (1988-1997) and identified 27 pat ients treated with DDAVP for 35 surgical events and 38 patients who receive d CFCs for 68 elective surgical events. Tranexamic acid was usually added f or mucosal surgery. The FVIII:C levels and the severity of surgery were use d 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 betwe en 34 and 52 IU/kg preoperatively and between 23 and 37 IU/kg postoperative ly. The effectiveness of haemostasis was excellent in 32 events (91%) treat ed with DDAVP and in 56 events (82%) treated with CFCs. It is concluded tha t patients with VWD do not carry an increased operative risk if appropriate therapy is given. Am. J. Hematol. 66:280-284, 2001. (C) 2001 Wiley-Liss, I nc.