Seminars in Hematology
Volume 43 , Pages S48-S58 , January 2006

Acquired von Willebrand Syndrome: An Underdiagnosed and Misdiagnosed Bleeding Complication in Patients With Lymphoproliferative and Myeloproliferative Disorders

  • Augusto B. Federici

      Affiliations

    • Corresponding Author InformationAddress correspondence to Augusto B. Federici, MD, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Regina Elena and University of Milan, Via Pace 9, 20122 Milano, Italy.

References 

  1. Mohri H , Motomura S , Kanamori H , Matsuzaki M , Watanabe S , Maruta A , et al.  Clinical significance of inhibitors in acquired von Willebrand syndrome . Blood . 1998;91:3623–3629
  2. Simone JV , Cornet JA , Abildgaard CF . Acquired von Willebrand’s syndrome in systemic lupus erythematosus . Blood . 1968;31:806–812
  3. Jakway JL . Acquired von Willebrand’s disease . Hematol Oncol Clin North Am . 1992;6:1409–1419
  4. van Genderen PJJ , Leenknegt H , Michiels JJ , Budde U . Acquired von Willebrand disease in myeloproliferative disorders . Leuk Lymphoma . 1996;22(suppl 1):79–82
  5. Rinder MR , Richard RE , Rinder HM . Acquired von Willebrand’s disease (A concise review) . Am J Hematol . 1997;54:139–145
  6. Tefferi A , Nichols WL . Acquired von Willebrand disease (Concise review of occurrence, diagnosis, pathogenesis and treatment) . Am J Med . 1997;103:536–540
  7. Nitu-Whalley IC , Lee CA . Acquired von Willebrand syndrome—Report of 10 cases and review of the literature . Haemophilia . 1999;5:318–326
  8. Federici AB . Therapeutic approaches to acquired von Willebrand syndrome . Expert Opin Invest Drugs . 2000;9:347–354
  9. Veyradier A , Jenkins CS , Fressinaud E , Meyer D . Acquired von Willebrand syndrome (From pathophysiology to management) . Thromb Haemost . 2000;84:175–182
  10. 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 Hematol . 2001;14:401–436
  11. Budde U , Bergmann F , Michiels  . Acquired von Willebrand syndrome (experience from 2 years in a single laboratory compared with data from the literature and an international registry) . Semin Thromb Hemost . 2002;28:227–238
  12. Federici AB , Rand JH , Bucciarelli P , Budde U , van Genderen PJ , Mohri H , et al.  Subcommittee on von Willebrand Factor: Acquired von Willebrand syndrome: Data from an international registry . Thromb Haemost . 2000;84:345–349
  13. Mannucci PM , Lombardi R , Bader R , Horellou MH , Finazzi G , Besana C , et al.  Studies of the pathophysiology of acquired von Willebrand’s disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies . Blood . 1984;64:614–621
  14. van Genderen PJJ , Boertjes RC , van Mourik JA . Quantitative analysis of von Willebrand factor and its propeptide in plasma in acquired von Willebrand syndrome . Thromb Haemost . 1998;80:495–498
  15. Federici AB , Berkowitz SD , Zimmerman TS , Mannucci PM . Proteolysis of von Willebrand factor after thrombolytic therapy in patients with acute myocardial infarction . Blood . 1992;79:38–44
  16. Federici AB , Berkowitz SD , Lattuada A , Mannucci PM . Degradation of von Willebrand factor in patients with acquired clinical conditions in which there is heightened proteolysis . Blood . 1993;81:720–725
  17. Mannucci PM , Federici AB . Antibodies to von Willebrand factor in von Willebrand disease . In:  Aledort LM ,  Hoyer LW ,  Reisner HM ,  White GC editor. Inhibitors to Coagulation Factors . New York: Plenum Press; 1995;p. 87–92
  18. Siaka C , Rugeri L , Caron C , Goudemand J . A new ELISA assay for diagnosis of acquired von Willebrand Syndrome . Haemophilia . 2003;9:303–308
  19. Noronha PA , Hruby MA , Maurer HS . Acquired von Willebrand disease in a patient with Wilms tumor . J Pediatr . 1979;95:997–999
  20. Scott JP , Montgomery RR , Tubergen DG , Hays T . Acquired von Willebrand’s disease in association with Wilm’s tumor (Regression following treatment) . Blood . 1981;58:665–669
  21. Richard C , Sedano MC , Cuadrado MA , Recio M , Hermosa V , Zubizarreta A . Acquired von Willebrand’s syndrome associated with hydatid disease of the spleen—Disappearance after splenectomy . Thromb Haemost . 1984;52:90–93
  22. Cappell MS , Lebwohl O . Cessation of recurrent bleeding from gastrointestinal angiodysplasia after aortic valve replacement . Ann Intern Med . 1986;105:54–57
  23. Weinstein M , Ware JA , Troll JH , Salzman EW . Changes in von Willebrand factor during cardiac surgery (Effect of desmopressin acetate) . Blood . 1988;71:1648–1655
  24. Coppes MJ , Zandvoort SWH , Sparling CR , Poon AO , Weitzman S , Blanchette VS . Acquired von Willebrand disease in Wilms’ tumor patients . J Clin Oncol . 1992;10:422–427
  25. Facon T , Caron C , Courtin P , Wurtz A , Deghaye M , Bauters F , et al.  Acquired type II von Willebrand’s disease associated with adrenal cortical carcinoma . Br J Haematol . 1992;80:488–494
  26. Anderson RP , McGrath K , Street A . Reversal of aortic stenosis, bleeding gastrointestinal angiodysplasia, and von Willebrand syndrome by aortic valve replacement . Lancet . 1996;347:689–690
  27. Pareti FI , Lattuada A , Bressi C , Zanobini M , Sala A , Steffan A , et al.  Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis . Circulation . 2000;102:1290–1295
  28. Vincentelli A , Susen S , Le Tourneau T , Six I , Fabre O , Juthier F , et al.  Acquired von Willebrand syndrome in aortic stenosis . N Engl J Med . 2003;349:343–349
  29. Duran-Saurez JR , Pico M , Zuazu J , Cortes M , Monasterio J , Tringiner J . Acquired von Willebrand’s disease caused by a chronic granulocytic leukemia . Br J Haematol . 1981;48:173–175
  30. Tran-Thang C , Mannucci PM , Schneider P , Federici AB , Bachmann F . Profound alterations of the multimeric structure of von Willebrand factor in a patient with malignant lymphoma . Br J Haematol . 1985;61:307–314
  31. Murakawa M , Okamura T , Tsutsumi K , Tanoguchi S , Kamura T , Shibuya T , et al.  Acquired von Willebrand’s disease in association with essential thrombocythemia (Regression following treatment) . Acta Haematol . 1992;87:83–87
  32. Budde U , van Genderen PJJ . Acquired von Willebrand disease in patients with high platelet counts . Semin Thromb Hemost . 1997;23:425–431
  33. Joist JH , Cowan JF , Zimmerman TS . Acquired von Willebrand’s disease. Evidence for a quantitative and qualitative factor VIII disorder . N Engl J Med . 1978;298:988–991
  34. Dalrymple-Hay M , Aitchison R , Collins R , Sekhar M , Colvin B . Hydroxyethyl starch induced acquired von Willebrand’s disease . Clin Lab Haematol . 1992;14:209–211
  35. Castaman G , Lattuada A , Mannucci PM , Rodeghiero F . Characterization of two cases of acquired transitory von Willebrand syndrome with ciprofloxacin (Evidence for heightened proteolysis of von Willebrand factor) . Am J Hematol . 1995;49:83–86
  36. Federici AB , Stabile F , Castaman G , Canciani MT , Mannucci PM . Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance (comparison of three different therapeutic approaches) . Blood . 1998;92:2707–2711
  37. Imbach PA , Kuhne T , Hollander G . Immunologic aspects in the pathogenesis and treatment of immune thrombocytopenic purpura in children . Curr Opin Pediatr . 1997;9:35–40
  38. van Genderen PJJ , Terpstra W , Michiels JJ , Kapteijn L , van Vliet HH . High-dose intravenous immunoglobin delays clearance of von Willebrand factor in acquired von Willebrand disease . Thromb Haemost . 1995;73:891–892
  39. Kazatchkine MD , Kaveri SV . Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin . N Engl J Med . 2001;345:747–755
  40. 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;66:292–294
  41. Ingram GIC , Kingston PJ , Leslie J , Bowie EJW . Four cases of acquired von Willebrand’s syndrome . Br J Haematol . 1971;21:189–199
  42. Budde U , Schaefer G , Mueller N , Egli H , Dent J , Ruggeri Z , et al.  Acquired von Willebrand’s disease in the myeloproliferative syndrome . Blood . 1984;64:981–985
  43. Budde U , Dent JA , Berkowitz SD , Ruggeri ZM , Zimmerman TS . Subunit composition of plasma von Willebrand factor in patients with the myeloproliferative syndrome . Blood . 1986;68:1213–1217
  44. 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;67:758–761
  45. Pickering NJ , Brody JI , Barrett MJ . von Willebrand syndromes and mitral-valve prolapse (Linked mesenchymal dysplasias) . N Engl J Med . 1981;305:131–134
  46. Rauch R , Budde U , Koch A , Girisch M , Hofbeck M . Acquired von Willebrand syndrome in children with patent ductus arteriosus . Heart . 2002;8:87–88
  47. Strauss RG , Stump DC , Henriksen RA . Hydroxyethyl starch accentuates von Willebrand’s disease . Transfusion . 1985;25:235–237
  48. Kreuz W , Linde R , Funk M , Meyer-Schrod R , Foll E , Nowak-Gottl U , et al.  Induction of von Willebrand disease type I by valproic acid . Lancet . 1990;335:1350–1351
  49. Conrad ME , Latour LF . Acquired von Willebrand’s disease, IgE polyclonal gammopathy and griseofulvin therapy . Am J Hematol . 1992;41:143–144
  50. Castaman G , Rodeghiero F . Acquired transitory von Willebrand syndrome with ciprofloxacin . Lancet . 1994;343:492
  51. Kinoshita S , Yoshioka K , Kasahara M , Takamiya O . Acquired von Willebrand disease after Epstein-Barr virus infection . J Pediatr . 1991;119:595–598
  52. Rogers JS , Shane SR , Jencks FS . Factor VIII activity and thyroid function . Ann Intern Med . 1982;97:713–716
  53. Mannucci PM , Remuzzi G , Pusineri F , Lombardi R , Valsecchi C , Mecca G , et al.  Deamino-8-D-arginine vasopressin shortens the bleeding time in uremia . N Engl J Med . 1983;308:8–12
  54. Brody JI , Levison SP , Jung CJ . Sickle cell trait and hematuria associated with von Willebrand syndromes . Ann Intern Med . 1977;86:529–533
  55. McGrath KM , Johnson CA , Stuart JJ . Acquired von Willebrand disease associated with an inhibitor to factor VIII antigen and gastrointestinal telangiectasia . Am J Med . 1979;67:693–696

 Supported in part by the Explorative Action on International Registry on AVWS grant given to A.B.F. by the University of Milan. Most of the data on the diagnosis and management of AVWS are updated from the International Registry on AVWS, and have been previously published on behalf of the Scientific Standardization Subcommittee (SSC) on VWF of the International Society on Thrombosis and Haemostasis (ISTH).

PII: S0037-1963(05)00210-6

doi: 10.1053/j.seminhematol.2005.11.003

Seminars in Hematology
Volume 43 , Pages S48-S58 , January 2006

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