Acquired von Willebrand disease—hemostatic management of major orthopedic surgery with high-dose immunoglobulin, desmopressin, and continuous factor concentrate infusion
Version of Record online: 30 APR 2002
Copyright © 2002 Wiley-Liss, Inc.
American Journal of Hematology
Volume 70, Issue 1, pages 64–71, May 2002
How to Cite
Frank, R. D., Kunz, D. and Wirtz, D. C. (2002), Acquired von Willebrand disease—hemostatic management of major orthopedic surgery with high-dose immunoglobulin, desmopressin, and continuous factor concentrate infusion. Am. J. Hematol., 70: 64–71. doi: 10.1002/ajh.10074
- Issue online: 30 APR 2002
- Version of Record online: 30 APR 2002
- Manuscript Accepted: 15 NOV 2001
- Manuscript Received: 22 JUN 2001
- acquired von Willebrand disease;
- high-dose immunoglobulin;
- von Willebrand factor;
- continuous infusion;
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 and exogenous VWF. High-dose intravenous immunoglobulin (ivIG) is recommended as the treatment of choice, usually rapidly normalizing coagulation; but in case of failure, alternative treatment options are not well explored. We report successful major orthopedic surgery in a 61-year-old woman with multiple myeloma IgG lambda and aVWD. IvIG alone failed to correct hemostasis. However, ivIG pretreatment improved the VWF ristocetin cofactor (VWF:RCo) half-life from only 1.5 hr to more than 4 hr, allowing desmopressin infusions twice daily to maintain sufficient VWF:RCo levels. Because of diminishing desmopressin effect, we attempted for the first time in aVWD a continuous VWF/FVIII infusion (Haemate HS®, 2.1–2.7 FVIII U/kg/hr or 51–64 U/kg/day, respectively 4.6–6.0 VWF:RCo U/kg/hr or 110–145 U/kg/day) to reach constant factor levels. The steady-state clearance was 2.4 mL/kg/hr for FVIII:C and 13.5 mL/kg/hr for VWF:RCo. During surgery, VWF:RCo, FVIII:C, and PFA-100 closure time were normalized. Until day 5, VWF:RCo was kept above 50%, from day 6 to 10 at least 30% activity were attained. FVIII:C levels were always >70%. The clinical course was uneventful without bleeding. Two weeks after hip surgery the patient was discharged from the hospital without complaints. The therapy described can be recommended as safe and feasible for further evaluation in aVWD patients who are hyporesponsive to ivIG treatment alone. Continuous VWF/FVIII infusion can improve substitution therapy in aVWD. Am. J. Hematol. 70:64–71, 2002. © 2002 Wiley-Liss, Inc.