Unexpected bleeding in the operating room: The role of acquired von Willebrand disease

Anesthesia and Analgesia

Volumn 114, Issue 1, 2012, Pages 73-81

  Lison, Susanne ^a   Dietrich, Wulf ^a   Spannagl, Michael ^a  

^a UNIVERSITY OF MUNICH   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING FACTOR 8 CONCENTRATE; COLLAGEN BINDING PROTEIN; CORTICOSTEROID; DESMOPRESSIN; IMMUNOGLOBULIN; IMMUNOGLOBULIN G; PREDNISONE; RECOMBINANT BLOOD CLOTTING FACTOR 7A; RISTOCETIN; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE; HEMOSTATIC AGENT;

ADULT; ANTIDIURESIS; AUTOSOMAL DOMINANT INHERITANCE; AUTOSOMAL RECESSIVE INHERITANCE; BLEEDING; BLEEDING TENDENCY; BLOOD CELL COUNT; BONE MARROW BIOPSY; CARDIAC PATIENT; CASE REPORT; CIRCULATION; CONTINUOUS INFUSION; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DISEASE SEVERITY; DRUG EFFICACY; DRUG MEGADOSE; DRUG SAFETY; EPISTAXIS; FOLLOW UP; GASTROINTESTINAL HEMORRHAGE; HEMOSTASIS; HERNIOTOMY; HOSPITALIZATION; HUMAN; HUMAN TISSUE; IMMUNOMODULATION; INTRAOPERATIVE PERIOD; ISCHIALGIA; MALE; MEDICAL HISTORY; MEGAKARYOCYTE; MENORRHAGIA; MONOCLONAL IMMUNOGLOBULINEMIA; NUCLEAR MAGNETIC RESONANCE IMAGING; OPERATING ROOM; PARTIAL THROMBOPLASTIN TIME; PEROPERATIVE COMPLICATION; PLASMAPHERESIS; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN FUNCTION; PROTHROMBIN TIME; REVIEW; SHEAR STRESS; SIDE EFFECT; SKIN BRUISING; THROMBOCYTE AGGREGATION; THROMBOCYTE FUNCTION; THROMBOGENESIS; TONSILLECTOMY; TOOTH EXTRACTION; TREATMENT DURATION; TREATMENT INDICATION; VON WILLEBRAND DISEASE; WOUND HEALING; BLOOD; BLOOD CLOTTING TEST; METABOLISM; MIDDLE AGED; RISK ASSESSMENT; RISK FACTOR; SURGERY;

BLOOD COAGULATION TESTS; BLOOD LOSS, SURGICAL; HEMOSTASIS; HEMOSTATICS; HUMANS; MALE; MIDDLE AGED; RISK ASSESSMENT; RISK FACTORS; SURGICAL PROCEDURES, OPERATIVE; VON WILLEBRAND DISEASES; VON WILLEBRAND FACTOR;

EID: 84655176754     PISSN: 00032999     EISSN: None     Source Type: Journal    
DOI: 10.1213/ANE.0b013e318236b16a     Document Type: Review

References (85)

1. Velik-Salchner C, Eschertzhuber S, Streif W, Hangler H, Budde U, Fries D. Acquired von Willebrand syndrome in cardiac patients. J Cardiothorac Vasc Anesth 2008;22:719-24
2. Veyradier A, Jenkins CS, Fressinaud E, Meyer D. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost 2000;84:175-82 (Pubitemid 30601460)
3. Tefferi A, Nichols WL. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis, and treatment. Am J Med 1997;103:536-40 (Pubitemid 28018657)
4. Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand's syndrome: a single institution experience. Am J Hematol 2003;72:243-7 (Pubitemid 36383974)
5. Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL, Rick ME, Sadler JE, Weinstein M, Yawn BP. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008;14:171-232 (Pubitemid 351343618)
6. German JW, Adamo MA, Hoppenot RG, Blossom JH, Nagle HA. Perioperative results following lumbar discectomy: comparison of minimally invasive discectomy and standard micro-discectomy. Neurosurg Focus 2008;25:E20
7. Sadler JE. Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem 1998;67:395-424 (Pubitemid 28411134)
8. Reininger AJ. Function of von Willebrand factor in haemostasis and thrombosis. Haemophilia 2008;14:11-26
9. Wise RJ, Dorner AJ, Krane M, Pittman DD, Kaufman RJ. The role of von Willebrand factor multimers and propeptide cleavage in binding and stabilization of factor VIII. J Biol Chem 1991;266:21948-55 (Pubitemid 121000273)
10. Weiss HJ, Sussman II, Hoyer LW. Stabilization of factor VIII in plasma by the von Willebrand factor: studies on posttransfusion and dissociated factor VIII and in patients with von Willebrand's disease. J Clin Invest 1977;60:390-404 (Pubitemid 8154146)
11. Dong JF. Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions. J Thromb Haemost 2005;3:1710-6
12. Feys HB, Deckmyn H, Vanhoorelbeke K. ADAMTS13 in health and disease. Acta Haematol 2009;121:183-5
13. Moake J. Thrombotic thrombocytopenia purpura (TTP) and other thrombotic microangiopathies. Best Pract Res Clin Haematol 2009;22:567-76
14. Bowen DJ, Collins PW. Insights into von Willebrand factor proteolysis: clinical implications. Br J Haematol 2006;133:457-67
15. Sadler JE. A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1994;71:520-5 (Pubitemid 24100682)
16. Federici AB, Castaman G, Mannucci PM. Guidelines for the diagnosis and management of von Willebrand disease in Italy. Haemophilia 2002;8:607-21
17. Silwer J. von Willebrand's disease in Sweden. Acta Paediatr Scand Suppl. 1973 238 1-159
18. Budde U. Diagnosis of von Willebrand disease subtypes: implications for treatment. Haemophilia 2008;14:27-38
19. Simone JV, Cornet JA, Abildgaard CF. Acquired von Will-ebrand's syndrome in systemic lupus erythematosus. Blood 1968;31:806-12
20. van Genderen PJ, Michiels JJ. Acquired von Willebrand disease. Baillieres Clin Haematol 1998;11:319-30 (Pubitemid 29103547)
21. Nitu-Whalley IC, Lee CA. Acquired von Willebrand syndrome: report of 10 cases and review of the literature. Haemophilia 1999;5:318-26 (Pubitemid 29495708)
22. Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H, Meyer D, Rodeghiero F, Sadler JE. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000;84:345-9 (Pubitemid 30601485)
23. Franchini M, Lippi G. Recent acquisitions in acquired and congenital von Willebrand disorders. Clin Chim Acta 2007;377:62-9 (Pubitemid 44908263)
24. Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F, Bauters A, Decoene C, Goudemand J, Prat A, Jude B. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003;349:343-9 (Pubitemid 36886240)
25. Pareti FI, Lattuada A, Bressi C, Zanobini M, Sala A, Steffan A, Ruggeri ZM. Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis. Circulation 2000;102:1290-5
26. Gill JC, Wilson AD, Endres-Brooks J, Montgomery RR. Loss of the largest von Willebrand factor multimers from the plasma of patients with congenital cardiac defects. Blood 1986;67:758-61 (Pubitemid 16130269)
27. Arslan MT, Ozyurek R, Kavakli K, Levent E, Ulger Z, Gurses D, Akyol B, Atay Y. Frequency of acquired von Willebrand's disease in children with congenital heart disease. Acta Cardiol 2007;62:403-8 (Pubitemid 47310627)
28. Heilmann C, Geisen U, Beyersdorf F, Nakamura L, Benk C, Berchtold-Herz M, Trummer G, Schlensak C, Zieger B. Acquired von Willebrand syndrome in patients with ventricular assist device or total artificial heart. Thromb Haemost 2010;103:962-7
29. Geisen U, Heilmann C, Beyersdorf F, Benk C, Berchtold-Herz M, Schlensak C, Budde U, Zieger B. Non-surgical bleeding in patients with ventricular assist devices could be explained by acquired von Willebrand disease. Eur J Cardiothorac Surg 2008;33:679-84
30. Manfredi E, van Zaane B, Gerdes VE, Brandjes DP, Squizzato A. Hypothyroidism and acquired von Willebrand's syndrome: a systematic review. Haemophilia 2008;14:423-33 (Pubitemid 351638727)
31. Jonville-Bera AP, Autret-Leca E, Gruel Y. Acquired type I von Willebrand's disease associated with highly substituted hy-droxyethyl starch. N Engl J Med 2001;345:622-3 (Pubitemid 32772113)
32. Kozek-Langenecker SA. Effects of hydroxyethyl starch solutions on hemostasis. Anesthesiology 2005;103:654-60 (Pubitemid 41243429)
33. Favaloro EJ, Lillicrap D, Lazzari MA, Cattaneo M, Mazurier C, Woods A, Meschengieser S, Blanco A, Kempfer AC, Hubbard A, Chang A. von Willebrand disease: laboratory aspects of diagnosis and treatment. Haemophilia 2004;10:164-8 (Pubitemid 40045437)
34. Nichols WL, Rick ME, Ortel TL, Montgomery RR, Sadler JE, Yawn BP, James AH, Hultin MB, Manco-Johnson MJ, Weinstein M. Clinical and laboratory diagnosis of von Willebrand disease: a synopsis of the 2008 NHLBI/NIH guidelines. Am J Hematol 2009;84:366-70
35. Favaloro EJ. Clinical utility of the PFA-100. Semin Thromb Hemost 2008;34:709-33
36. Posan E, McBane RD, Grill DE, Motsko CL, Nichols WL. Comparison of PFA-100 testing and bleeding time for detecting platelet hypofunction and von Willebrand disease in clinical practice. Thromb Haemost 2003;90:483-90 (Pubitemid 37138774)
37. Koscielny J, Ziemer S, Radtke H, Schmutzler M, Pruss A, Sinha P, Salama A, Kiesewetter H, Latza R. A practical concept for preoperative identification of patients with impaired primary hemostasis. Clin Appl Thromb Hemost 2004;10:195-204 (Pubitemid 38982043)
38. Sucker C, Feindt P, Zotz RB, Stockschlaeder M, Scharf RE. Functional von Willebrand factor assays are not predictive for the absence of highest-molecular weight von Willebrand factor multimers in patients with aortic-valve stenosis. Thromb Hae-most 2005;94:465-6 (Pubitemid 41122506)
39. Tiede A, Priesack J, Werwitzke S, Bohlmann K, Oortwijn B, Lenting P, Eisert R, Ganser A, Budde U. Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study. J Thromb Haemost 2008;6: 569-76 (Pubitemid 351404923)
40. Yoshida K, Tobe S, Kawata M, Yamaguchi M. Acquired and reversible von Willebrand disease with high shear stress aortic valve stenosis. Ann Thorac Surg 2006;81:490-4 (Pubitemid 43120541)
41. Tsai HM, Sussman II, Nagel RL. Shear stress enhances the proteolysis of von Willebrand factor in normal plasma. Blood 1994;83:2171-9 (Pubitemid 24112728)
42. Rauch R, Budde U, Koch A, Girisch M, Hofbeck M. Acquired von Willebrand syndrome in children with patent ductus arteriosus. Heart 2002;88:87-8 (Pubitemid 34665016)
43. Malehsa D, Meyer AL, Bara C, Struber M. Acquired von Willebrand syndrome after exchange of the HeartMate XVE to the HeartMate II ventricular assist device. Eur J Cardiothorac Surg 2009;35:1091-3
44. Mikkelsen MM, Fenger-Eriksen C, Johnsen SP, Christensen TD, Sorensen B. No excess surgical blood loss in patients with acquired type 2A von Willebrand disease. Scand Cardiovasc J 2011;45:120-6
45. Morishima A, Marui A, Shimamoto T, Saji Y, Tambara K, Nishina T, Komeda M. Successful aortic valve replacement for Heyde syndrome with confirmed hematologic recovery. Ann Thorac Surg 2007;83:287-8 (Pubitemid 44917863)
46. Crow S, Chen D, Milano C, Thomas W, Joyce L, Piacentino V III, Sharma R, Wu J, Arepally G, Bowles D, Rogers J, Villamizar-Ortiz N. Acquired von Willebrand syndrome in continuous-flow ventricular assist device recipients. Ann Tho-rac Surg 2010;90:1263-9
47. Uriel N, Pak SW, Jorde UP, Jude B, Susen S, Vincentelli A, Ennezat PV, Cappleman S, Naka Y, Mancini D. Acquired von Willebrand syndrome after continuous-flow mechanical device support contributes to a high prevalence of bleeding during long-term support and at the time of transplantation. J Am Coll Cardiol 2010;56:1207-13
48. Michiels JJ, Budde U, van der Planken M, van Vliet HH, Schroyens W, Berneman Z. Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol 2001;14:401-36 (Pubitemid 32677087)
49. Federici AB. Therapeutic approaches to acquired von Willebrand syndrome. Expert Opin Investig Drugs 2000;9:347-54 (Pubitemid 30032316)
50. Castaman G, Rodeghiero F, Di Bona E, Ruggeri M. Clinical effectiveness of desmopressin in a case of acquired von Will-ebrand's syndrome associated with benign monoclonal gam-mopathy. Blut 1989;58:211-3 (Pubitemid 19122782)
51. Coppola A, Di Minno G. Desmopressin in inherited disorders of platelet function. Haemophilia 2008;14:31-9
52. Sloand EM, Alyono D, Klein HG, Chang P, Yu M, Lightfoot FG, Kessler C. 1-Deamino-8-D-arginine vasopressin (DDAVP) increases platelet membrane expression of glycoprotein Ib in patients with disorders of platelet function and after cardio-pulmonary bypass. Am J Hematol 1994;46:199-207 (Pubitemid 24206745)
53. Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand disease. Mayo Clin Proc 2002;77:181-7 (Pubitemid 34118895)
54. Alvarez MT, Jimenez-Yuste V, Gracia J, Quintana M, Hernandez-Navarro F. Acquired von Willebrand syndrome. Haemophilia 2008;14:856-8 (Pubitemid 352016050)
55. Mannucci PM. Treatment of von Willebrand's disease. N Engl J Med 2004;351:683-94
56. Van Genderen PJ, Papatsonis DN, Michiels JJ, Wielenga JJ, Stibbe J, Huikeshoven FJ. High-dose intravenous gam-maglobulin therapy for acquired von Willebrand disease. Postgrad Med J 1994;70:916-20
57. Batlle J, Lopez-Fernandez MF, Fraga EL, Trillo AR, Perez-Rodriguez MA. von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease. Blood Coagul Fibrinolysis 2009;20:89-100
58. Michiels JJ, Schroyens W, van der Planken M, Berneman Z. Acquired von Willebrand syndrome in systemic lupus erythe-matodes. Clin Appl Thromb Hemost 2001;7:106-12 (Pubitemid 32242185)
59. Silberstein LE, Abrahm J, Shattil SJ. The efficacy of intensive plasma exchange in acquired von Willebrand's disease. Transfusion 1987;27:234-7 (Pubitemid 17087589)
60. Federici AB. Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome. Hum Immunol 2005;66:422-30 (Pubitemid 40615799)
61. Hayashi T, Yagi H, Suzuki H, Nonaka Y, Nomura T, Sakurai Y, Shibata M, Matsumoto M, Yamamoto Y, Fujimura Y. Low-dosage intravenous immunoglobulin in the management of a patient with acquired von Willebrand syndrome associated with monoclonal gammopathy of undetermined significance. Pathophysiol Haemost Thromb 2002;32:33-9 (Pubitemid 37490485)
62. Michiels JJ, van Vliet HH. Acquired von Willebrand disease in monoclonal gammapathies: effectiveness of high-dose intravenous gamma globulin. Clin Appl Thromb Hemost 1999;5:152-7 (Pubitemid 29293535)
63. Eikenboom JC, Tjernberg P, Van Marion V, Heering KJ. Acquired von Willebrand syndrome: diagnostic problems and therapeutic options. Am J Hematol 2007;82:55-8 (Pubitemid 46033393)
64. Mohri H. Acquired von Willebrand syndrome: features and management. Am J Hematol 2006;81:616-23 (Pubitemid 44066172)
65. 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;70:64-71
66. Maddox JM, Anderson JA, Plews D, Ludlam CA. Management of acquired von Willebrand's syndrome in a patient requiring major surgery. Haemophilia 2005;11:633-7 (Pubitemid 41631600)
67. Lipkind HS, Kurtis JD, Powrie R, Carpenter MW. Acquired von Willebrand disease: management of labor and delivery with intravenous dexamethasone, continuous factor concentrate, and immunoglobulin infusion. Am J Obstet Gynecol 2005;192:2067-70 (Pubitemid 40827089)
68. Abshire T, Kenet G. Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors. J Thromb Haemost 2004;2:899-909 (Pubitemid 40185737)
69. Poon MC. The evidence for the use of recombinant human activated factor VII in the treatment of bleeding patients with quantitative and qualitative platelet disorders. Transfus Med Rev 2007;21:223-36
70. Franchini M, Zaffanello M, Veneri D. Recombinant factor VIIa: an update on its clinical use. Thromb Haemost 2005;93:1027-35 (Pubitemid 40823504)
71. Lisman T, De Groot PG. Mechanism of action of recombinant factor VIIa. J Thromb Haemost 2003;1:1138-9
72. Lisman T, Adelmeijer J, Heijnen HF, de Groot PG. Recombinant factor VIIa restores aggregation of alphaIIbbeta3-deficient platelets via tissue factor-independent fibrin generation. Blood 2004;103:1720-7 (Pubitemid 38268964)
73. Sucker C, Scharf RE, Zotz RB. Use of recombinant factor VIIa in inherited and acquired von Willebrand disease. Clin Appl Thromb Hemost 2009;15:27-31
74. Franchini M, Veneri D, Lippi G. The use of recombinant activated factor VII in congenital and acquired von Willebrand disease. Blood Coagul Fibrinolysis 2006;17:615-9 (Pubitemid 44760419)
75. Karger R, Weippert-Kretschmer M, Budde U, Kretschmer V. Diagnosis and therapeutic management in a patient with type 2B-like acquired von Willebrand syndrome. Blood Coagul Fibrinolysis 2011;22:144-7
76. Perez-Montes R, Arce O, Bermudez A, Yanez L, Sedano C, Zubizarreta A. Recombinant factor VIIa as a hemostatic agent in acquired von Willebrand disease. J Thromb Haemost 2003;1:1129
77. Friederich PW, Wever PC, Briet E, Doorenbos CJ, Levi M. Successful treatment with recombinant factor VIIa of therapy-resistant severe bleeding in a patient with acquired von Willebrand disease. Am J Hematol 2001;66:292-4 (Pubitemid 32210299)
78. Smaradottir A, Bona R. A case of acquired von Willebrand syndrome successfully treated with recombinant factor VIIa during thyroidectomy. Thromb Haemost 2004;92:666-7 (Pubitemid 39248026)
79. Basso IN, Keeling D. Myocardial infarction following recombinant activated factor VII in a patient with type 2A von Willebrand disease. Blood Coagul Fibrinolysis 2004;15:503-4 (Pubitemid 39180236)
80. Levi M, Levy JH, Andersen HF, Truloff D. Safety of recombinant activated factor VII in randomized clinical trials. N Engl J Med 2010;363:1791-800
81. Rinder MR, Richard RE, Rinder HM. Acquired von Willebrand's disease: a concise review. Am J Hematol 1997;54:139-45 (Pubitemid 27079420)
82. Hennessy BJ, White B, Byrne M, Smith OP. Acquired von Willebrand's disease. Ir J Med Sci 1998;167:81-5 (Pubitemid 28266296)
83. Sanchez-Luceros A, Meschengieser SS, Woods AI, Blanco AN, Kempfer AC, Casais P, Salviu MJ, Lazzari MA. Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution. Haematologica 2002;87:264-70 (Pubitemid 34212442)
84. Yoshida K, Tobe S, Kawata M. Acquired von Willebrand disease type IIA in patients with aortic valve stenosis. Ann Thorac Surg 2006;81:1114-6 (Pubitemid 43247705)
85. Sardesai MP, Sabik J, Koch CG. Perioperative management of acquired von Willebrand disease in cardiac surgery: type 2B or not 2B? J Cardiothorac Vasc Anesth 2011;25:131-3