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Acquired von Willebrand disease--hemostatic management of major orthopedic surgery with high-dose immunoglobulin, desmopressin, and continuous factor concentrate infusion.
Am J Hematol 2002; 70(1):64-71AJ

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 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.

Authors+Show Affiliations

Department of Nephrology and Clinical Immunology, University Hospital RWTH Aachen, Pauwelsstrasse 30, D-52057 Aachen, Germany. dario.frank@ukaachen.deNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

11994985

Citation

Frank, Rolf Dario, et al. "Acquired Von Willebrand Disease--hemostatic Management of Major Orthopedic Surgery With High-dose Immunoglobulin, Desmopressin, and Continuous Factor Concentrate Infusion." American Journal of Hematology, vol. 70, no. 1, 2002, pp. 64-71.
Frank RD, Kunz D, Wirtz DC. Acquired von Willebrand disease--hemostatic management of major orthopedic surgery with high-dose immunoglobulin, desmopressin, and continuous factor concentrate infusion. Am J Hematol. 2002;70(1):64-71.
Frank, R. D., Kunz, D., & Wirtz, D. C. (2002). Acquired von Willebrand disease--hemostatic management of major orthopedic surgery with high-dose immunoglobulin, desmopressin, and continuous factor concentrate infusion. American Journal of Hematology, 70(1), pp. 64-71.
Frank RD, Kunz D, Wirtz DC. Acquired Von Willebrand Disease--hemostatic Management of Major Orthopedic Surgery With High-dose Immunoglobulin, Desmopressin, and Continuous Factor Concentrate Infusion. Am J Hematol. 2002;70(1):64-71. PubMed PMID: 11994985.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acquired von Willebrand disease--hemostatic management of major orthopedic surgery with high-dose immunoglobulin, desmopressin, and continuous factor concentrate infusion. AU - Frank,Rolf Dario, AU - Kunz,Dagmar, AU - Wirtz,Dieter Christian, PY - 2002/5/8/pubmed PY - 2002/6/12/medline PY - 2002/5/8/entrez SP - 64 EP - 71 JF - American journal of hematology JO - Am. J. Hematol. VL - 70 IS - 1 N2 - 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. SN - 0361-8609 UR - https://www.unboundmedicine.com/medline/citation/11994985/Acquired_von_Willebrand_disease__hemostatic_management_of_major_orthopedic_surgery_with_high_dose_immunoglobulin_desmopressin_and_continuous_factor_concentrate_infusion_ L2 - https://doi.org/10.1002/ajh.10074 DB - PRIME DP - Unbound Medicine ER -