The Role Of von Willebrand Factor In Hemorrhagic And Thrombotic Disorders

REFERENCES

• Sadler J E. von Willebrand factor: two sides of a coin. J Thromb Haemost 2005; 3: 1702–1709
   PubMed Web of Science ®Google Scholar
• Martinelli I. von Willebrand factor and factor VIII as risk factors for arterial and venous thrombosis. Semin Hematol 2005; 42: 49–55
   PubMed Web of Science ®Google Scholar
• Soejima K, Nakagaki T. Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies. Semin Hematol 2005; 42: 56–62
   PubMed Web of Science ®Google Scholar
• Castaman G, Federici A B, Rodeghiero F, Mannucci P M. von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica 2003; 88: 94–108
   PubMed Web of Science ®Google Scholar
• Mancuso D J, Tuley E A, Westfield L A, Lester-Mancuso T L, Le Beau M M, Sorace J M, Sadler J E. Human von Willebrand factor gene and pseudogene: structural analysis and differentiation by polymerase chain reaction. Biochemistry 1991; 30: 253–269
   PubMed Web of Science ®Google Scholar
• Sadler J E, Mancuso D J, Randi A M, Tuley E A, Westfield L A. Molecular biology of von Willebrand factor. Ann NY Acad Sci 1991; 614: 114–124
   PubMed Web of Science ®Google Scholar
• Mendolicchio G L, Ruggeri Z M. New perspectives on von Willebrand factor functions in hemostasis and thrombosis. Semin Hematol 2005; 42: 5–14
   PubMed Web of Science ®Google Scholar
• Wagner D D. Cell biology of von Willebrand factor. Annu Rev Cell Biol 1990; 6: 217–246
   PubMedGoogle Scholar
• Ruggeri Z M. von Willebrand factor. Curr Opin Hematol 2003; 10: 142–149
   PubMed Web of Science ®Google Scholar
• Dong J F, Moake J L, Nolasco L, Bernardo A, Arceneaux W, Shrimpton C N, Schade A J, McIntire L V, Fujikawa K, Lopez J A. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood 2002; 100: 4033–4039
   PubMed Web of Science ®Google Scholar
• Schmugge M, Rand M L, Freedman J. Platelets and von Willebrand factor. Transfus Apheresis Sci 2003; 28: 269–277
   PubMed Web of Science ®Google Scholar
• Ruggeri Z M. Structure of von Willebrand factor and its function in platelet adhesion and thrombus formation. Best Pract Res Clin Haematol 2001; 14: 257–279
   PubMed Web of Science ®Google Scholar
• Ruggeri Z M. Old concepts and new developments in the study of platelet aggregation. J Clin Invest 2000; 105: 699–701
   PubMed Web of Science ®Google Scholar
• Vlot A J, Koppelman S J, Bouma B N, Sixma J J. Factor VIII and von Willebrand factor. Thromb Haemost 1998; 79: 456–465
   PubMed Web of Science ®Google Scholar
• Gill J C, Endres-Brooks J, Bauer P J, Marks W J, Jr., Montgomery R R. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987; 69: 1691–1695
   PubMed Web of Science ®Google Scholar
• Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood 1987; 69: 454–459
   PubMed Web of Science ®Google Scholar
• Federici A B, Castaman G. Mannucci PM; Italian Association of Hemophilia Centers (AICE). Guidelines for the diagnosis and management of von Willebrand disease in Italy. Haemophilia 2002; 8: 607–621
   PubMed Web of Science ®Google Scholar
• Mammen E F. Laboratory issues in the identification and diagnosis of von Willebrand disease on the 80th anniversary of Erik von Willebrand's original publication. Semin Thromb Hemost 2006; 32: 443–444
   PubMed Web of Science ®Google Scholar
• Federici A B. Clinical diagnosis of von Willebrand disease. Haemophilia 2004; 10: 169–176, (Suppl 4)
   PubMed Web of Science ®Google Scholar
• Miller C H, Graham J B, Goldin L R, Elston R C. Genetics of classic von Willebrand's disease. I. Phenotypic variation within families. Blood 1979; 54: 117–136
   PubMed Web of Science ®Google Scholar
• Weiss H J, Pietu G, Rabinowitz R, Girma J P, Rogers J, Meyer D. Heterogeneous abnormalities in the multimeric structure, antigenic properties, and plasma-platelet content of factor VIII/von Willebrand factor in subtypes of classic (type I) and variant (IIA) von Willebrand's disease. J Lab Clin Med 1983; 101: 411–425
   PubMed Web of Science ®Google Scholar
• Mannucci P M, Lombardi R, Bader R, Vianello L, Federici A B, Solinas S, Mazzucconi M G, Mariani G. Heterogeneity of type I von Willebrand disease: evidence for a subgroup with an abnormal von Willebrand factor. Blood 1985; 66: 796–802
   PubMed Web of Science ®Google Scholar
• Favaloro E J, Lillicrap D, Lazzari M A, Cattaneo M, Mazurier C, Woods A, Meschengieser S, Blanco A, Kempfer A C, Hubbard A, Chang A. von Willebrand disease: laboratory aspects of diagnosis and treatment. Haemophilia 2004; 10: 164–168, (Suppl 4)
   PubMed Web of Science ®Google Scholar
• Laffan M, Brown S A, Collins P W, Cumming A M, Hill F G, Keeling D, Peake I R, Pasi K J. The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004; 10: 199–217
   PubMed Web of Science ®Google Scholar
• Weiss H J, Meyer D, Rabinowitz R, Pietu G, Girma J P, Vicic W J, Rogers J. Pseudo-von Willebrand's disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII/von Willebrand factor and enhanced adsorption of its high-molecular-weight multimers. N Engl J Med 1982; 306: 326–333
   PubMed Web of Science ®Google Scholar
• Miller J L, Castella A. Platelet-type von Willebrand's disease: characterization of a new bleeding disorder. Blood 1982; 60: 790–794
   PubMed Web of Science ®Google Scholar
• Nishino M, Girma J P, Rothschild C, Fressinaud E, Meyer D. New variant of von Willebrand disease with defective binding to factor VIII. Blood 1989; 74: 1591–1599
   PubMed Web of Science ®Google Scholar
• Mazurier C, Dieval J, Jorieux S, Delobel J, Goudemand M. A new von Willebrand factor (VWF) defect in a patient with factor VIII (FVIII) deficiency but with normal levels and multimeric patterns of both plasma and platelet VWF. Characterization of abnormal VWF/FVIII interaction. Blood 1990; 75: 20–26
   PubMed Web of Science ®Google Scholar
• Franchini M. The platelet function analyzer (PFA-100): an update on its clinical use. Clin Lab 2005; 51: 367–372
   PubMed Web of Science ®Google Scholar
• Federici A B, Canciani M T, Forza I, Cozzi G. Ristocetin cofactor and collagen binding activities normalized to antigen levels for a rapid diagnosis of type 2 von Willebrand disease—single center comparison of four different assays. Thromb Haemost 2000; 84: 1127–1128
   PubMed Web of Science ®Google Scholar
• Federici A B, de Groot P G, Moia M, Ijsseldijk M J, Sixma J J, Mannucci P M. Type I von Willebrand disease, subtype ‘platelet low’: decreased platelet adhesion can be explained by low synthesis of von Willebrand factor in endothelial cells. Br J Haematol 1993; 83: 88–93
   PubMed Web of Science ®Google Scholar
• Sadler J E. A revised classification of von Willebrand disease. Thromb Haemost 1994; 71: 520–525
   PubMed Web of Science ®Google Scholar
• Meyer D, Fressinaud E, Gaucher C, Lavergne J M, Hilbert L, Ribba A S, Jorieux S, Mazurier C. Gene defects in 150 unrelated French cases with type 2 von Willebrand disease: from the patient to the gene. INSERM Network on Molecular Abnormalities in von Willebrand Disease. Thromb Haemost 1997; 78: 451–456
   PubMed Web of Science ®Google Scholar
• Lyons S E, Bruck M E, Bowie E J, Ginsburg D. Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations. J Biol Chem 1992; 267: 4424–4430
   PubMed Web of Science ®Google Scholar
• Schneppenheim R, Budde U, Ruggeri Z M. A molecular approach to the classification of von Willebrand disease. Best Pract Res Clin Haematol 2001; 14: 281–298
   PubMed Web of Science ®Google Scholar
• Sadler J E. New concepts in von Willebrand disease. Annu Rev Med 2005; 56: 173–191
   PubMed Web of Science ®Google Scholar
• Ruggeri Z M. Type IIB von Willebrand disease: a paradox explains how von Willebrand factor works. J Thromb Haemost 2004; 2: 2–6
   PubMed Web of Science ®Google Scholar
• Savage B, Saldivar E, Ruggeri Z M. Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. Cell 1996; 84: 289–297
   PubMed Web of Science ®Google Scholar
• Miura S, Li C Q, Cao Z, Wang H, Wardell M R, Sadler J E. Interaction of von Willebrand factor domain A1 with platelet glycoprotein Ibalpha-(1-289). Slow intrinsic binding kinetics mediate rapid platelet adhesion. J Biol Chem 2000; 275: 7539–7546
   PubMed Web of Science ®Google Scholar
• Mancuso D J, Kroner P A, Christopherson P A, Vokac E A, Gill J C, Montgomery R R. Type 2M:Milwaukee-1 von Willebrand disease: an in-frame deletion in the Cys509-Cys695 loop of the von Willebrand factor A1 domain causes deficient binding of von Willebrand factor to platelets. Blood 1996; 88: 2259–2568
   PubMed Web of Science ®Google Scholar
• Hillery C A, Mancuso D J, Evan Sadler J, Ponder J W, Jozwiak M A, Christopherson P A, Cox Gill J, Paul Scott J, Montgomery R R. Type 2M von Willebrand disease: F606I and I662F mutations in the glycoprotein Ib binding domain selectively impair ristocetin-but not botrocetin-mediated binding of von Willebrand factor to platelets. Blood 1998; 91: 1572–1581
   PubMed Web of Science ®Google Scholar
• Mannucci P M, Lombardi R, Castaman G, Dent J A, Lattuada A, Rodeghiero F, Zimmerman T S. von Willebrand disease “Vicenza” with larger-than-normal (supranormal) von Willebrand factor multimers. Blood 1988; 71: 65–70
   PubMed Web of Science ®Google Scholar
• Schneppenheim R, Federici A B, Budde U, Castaman G, Drewke E, Krey S, Mannucci P M, Riesen G, Rodeghiero F, Zieger B, Zimmermann R. Von Willebrand disease type 2M “Vicenza” in Italian and German patients: identification of the first candidate mutation (G3864A; R1205H) in 8 families. Thromb Haemost 2000; 82: 136–140
   Google Scholar
• Mazurier C, Goudemand J, Hilbert L, Caron C, Fressinaud E, Meyer D. Type 2N von Willebrand disease: clinical manifestations, pathophysiology, laboratory diagnosis and molecular biology. Best Pract Res Clin Hematol 2001; 14: 337–347
   PubMed Web of Science ®Google Scholar
• Sadler J E, Mannucci P M, Berntorp E, Bochkov N, Boulyjenkov V, Ginsburg D, Meyer D, Peake I, Rodeghiero F, Srivastava A. Impact, diagnosis and treatment of von Willebrand disease. Thromb Haemost 2000; 84: 160–174
   PubMed Web of Science ®Google Scholar
• Mannucci P M, Ruggeri Z M, Ciavarella N, Kazatchkine M D, Mowbray J F. Precipitating antibodies to factor VIII/von Willebrand factor in von Willebrand's disease: effects on replacement therapy. Blood 1981; 57: 25–31
   PubMed Web of Science ®Google Scholar
• Pasi K J, Collins P W, Keeling D M, Brown S A, Cumming A M, Dolan G C, Hay C R, Hill F G, Laffan M, Peake I R. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004; 10: 218–231
   PubMed Web of Science ®Google Scholar
• Mannucci P M. Treatment of von Willebrand's disease. N Engl J Med 2004; 351: 683–694
   PubMed Web of Science ®Google Scholar
• Mannucci P M, Federici A B. Management of inherited von Willebrand disease. Best Pract Res Clin Haematol 2001; 14: 455–462
   PubMed Web of Science ®Google Scholar
• Rodeghiero F, Castaman G, Mannucci P M. Clinical indications for desmopressin (DDAVP) in congenital and acquired von Willebrand disease. Blood Rev 1991; 5: 155–161
   PubMed Web of Science ®Google Scholar
• Mannucci P M. How I treat patients with von Willebrand disease. Blood 2001; 97: 1915–1919
   PubMed Web of Science ®Google Scholar
• Mannucci P M. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first twenty years. Haemophilia 2000; 6: 60–67, (Suppl 1)
   PubMed Web of Science ®Google Scholar
• Mannucci P M, Chediak J, Hanna W, Byrnes J, Marlies L, Ewenstein B M. The Alphanate Study Group. Treatment of von Willebrand disease with a high-purity factor VIII/von Willebrand factor concentrate: a prospective, multicenter study. Blood 2002; 99: 450–456
   PubMed Web of Science ®Google Scholar
• Franchini M, Rossetti G, Tagliaferri A, Pattacini C, Pozzoli D, Lippi G, Manzato F, Bertuzzo D, Gandini G. Efficacy and safety of factor VIII/von Willebrand factor concentrate (Haemate-P) in preventing bleeding during surgery or invasive procedures in patients with von Willebrand's disease. Haematologica 2003; 88: 1279–1283
   PubMed Web of Science ®Google Scholar
• Rosendaal F R. High levels of factor VIII and venous thrombosis. Thromb Haemost 2000; 83: 1–2
   PubMed Web of Science ®Google Scholar
• Mannucci P M. Venous thromboembolism in von Willebrand disease. Thromb Haemost 2002; 88: 378–379
   PubMed Web of Science ®Google Scholar
• Makris M, Colvin B, Gupta V, Shields M L, Smith M P. Venous thrombosis following the use of intermediate purity FVIII concentrate to treat patients with von Willebrand's disease. Thromb Haemost 2002; 88: 387–388
   PubMed Web of Science ®Google Scholar
• Rodeghiero F, Castaman G. Treatment of von Willebrand disease. Semin Hematol 2005; 42: 29–35
   PubMed Web of Science ®Google Scholar
• Mannucci P M, Tamaro G, Narchi G, Candotti G, Federici A, Altieri D, Tedesco F. Life-threatening reaction to factor VIII concentrate in a patient with severe von Willebrand disease and alloantibodies to von Willebrand factor. Eur J Haematol 1987; 39: 467–470
   PubMed Web of Science ®Google Scholar
• Shapiro S, Hultin M. Acquired inhibitors to coagulation factors. Semin Thromb Hemost 1975; 1: 336–385
   Google Scholar
• Handin R I, Martin V, Maloney W C. Antibody induced von Willebrand syndrome. A newly defined inhibitor syndrome. Blood 1976; 48: 393–405
   PubMed Web of Science ®Google Scholar
• Holland L, Adamson A, Ingram G IC, Chalmers D G. Acquired von Willebrand syndrome. Br J Haematol 1980; 45: 161–164
   PubMed Web of Science ®Google Scholar
• Bloom A L. The von Willebrand syndrome. Semin Hematol 1980; 27: 215–227
   Google Scholar
• Jakway J. Acquired von Willebrand's disease in malignancy. Semin Thromb Hemost 1992; 18: 434–439
   PubMed Web of Science ®Google Scholar
• Cohen A J, Kessler C M. Auto-antibody against inhibitors of von Willebrand factor protein. Bailliere's Clin Haematol 1996; 9: 348–354
   Google Scholar
• Rinder M R, Richard R E, Rinder H M. Acquired von Willebrand disease: a concise review. Am J Haematol 1997; 54: 139–145
   PubMed Web of Science ®Google Scholar
• Tefferi A, Nichols W L. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis and treatment. Am J Med 1997; 103: 536–540
   PubMed Web of Science ®Google Scholar
• Veyradier A, Jenkins C S, Fressinaud E, Meyer D. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost 2000; 84: 175–182
   PubMed Web of Science ®Google Scholar
• Mohri H. Acquired von Willebrand syndrome: its pathophysiology, laboratory features and management. J Thromb Thrombolysis 2003; 15: 141–149
   PubMed Web of Science ®Google Scholar
• Federici A B, Budde U, Rand J H. Acquired von Willebrand syndrome 2004: international registry. Hamostaseologie 2004; 24: 50–55
   PubMedGoogle Scholar
• Michiels J J, Budde U, van der Planken M, van Vliet H, Schroyens W, Berneman Z. Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol 2001; 14: 401–436
   PubMed Web of Science ®Google Scholar
• Lollar P. Pathogenic antibodies to coagulation factors. Part II. Fibrinogen, prothrombin, thrombin, factor V, factor XI, factor XII, factor XIII, the protein C system and von Willebrand factor. J Thromb Haemost 2005; 3: 1385–1391
   PubMed Web of Science ®Google Scholar
• Simone J V, Cornet J A, Abildgaard C F. Acquired von Willebrand's syndrome in systemic lupus erythematosus. Blood 1968; 31: 806–812
   PubMed Web of Science ®Google Scholar
• Federici A B, Rand J H, Bucciarelli P, Budde U, van Genderen P J, Mohri H, Meyer D, Rodeghiero F, Sadler J E. Subcommittee on von Willebrand Factor. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000; 84: 345–349
   PubMed Web of Science ®Google Scholar
• Budde U, Bergemann F, Michiels J J. 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
   PubMed Web of Science ®Google Scholar
• Mannucci P M, Lombardi R, Bader R. 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
   PubMed Web of Science ®Google Scholar
• Mant M J, Hirsh J, Gauldie J, Bienenstock J, Pineo J, Luke K H. Von Willebrand's syndrome presenting as an acquired bleeding disorder in association with a monoclonal gammopathy. Blood 1973; 42: 429–436
   PubMed Web of Science ®Google Scholar
• Stewart A K, Glynn M F. Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisolone. Postgrad Med J 1990; 66: 560–562
   PubMed Web of Science ®Google Scholar
• Federici A B, Stabile F, Castaman G, Canciani M T, Mannucci P M. Treatment of acquired von Willebrand disease in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood 1998; 92: 2707–2711
   PubMed Web of Science ®Google Scholar
• Nitu-Whalley I C, Lee C A. Acquired von Willebrand syndrome—report of 10 cases and review of the literature. Haemophilia 1999; 5: 318–326
   PubMed Web of Science ®Google Scholar
• Federici A B, Rand J H, Mannucci P M. Acquired von Willebrand syndrome: an important bleeding complication to be considered in patients with lymphoproliferative and myeloproliferative disorders. Hematol J 2001; 2: 358–362
   PubMedGoogle Scholar
• Maddox J M, Anderson J AM, Plews D, Ludlam C A. Management of acquired von Willebrand's syndrome in a patient requiring major surgery. Haemophilia 2005; 11: 633–637
   PubMed Web of Science ®Google Scholar
• Sampson B M, Greaves M, Malia R G, Preston F E. Acquired von Willebrand's disease: demonstration of circulating inhibitor to the FVIII complex in four cases. Br J Haematol 1983; 54: 233–244
   PubMed Web of Science ®Google Scholar
• Bovill E G, Ershler W B, Golden E A, Tindle B H, Edson J R. A human myeloma-produced monoclonal protein directed against the active subpopulation of von Willebrand factor. Am J Clin Pathol 1986; 85: 115–123
   PubMed Web of Science ®Google Scholar
• Richard C, Cuardo M A, Prieto M. Acquired von Willebrand disease myeloma secondary to absorption of von Willebrand factor by plasma cells. Am J Hematol 1990; 35: 114–117
   PubMed Web of Science ®Google Scholar
• Takahashi H, Nagayama R, Tanabe Y. DDAVP in acquired von Willebrand syndrome associated with multiple myeloma. Am J Hematol 1986; 22: 421–429
   PubMed Web of Science ®Google Scholar
• Mohri H, Noguchi T, Kodama F, Itoh A, Ohkubo T. Acquired von Willebrand disease due to inhibitor of human myeloma protein specific for von Willebrand factor. Am J Clin Pathol 1987; 87: 663–668
   PubMed Web of Science ®Google Scholar
• Mohri H, Tanabe J, Ohtsuka M, Yoshida M, Motomura S, Nishida S, Fujimura Y, Okubo T. Acquired von Willebrand disease associated with multiple myeloma; characterization of an inhibitor to von Willebrand factor. Blood Coagul Fibrinolysis 1995; 6: 561–566
   PubMed Web of Science ®Google Scholar
• Mazurier C, Parquet-Gernez A, Descamps J, Bauters F, Goudeman M. Acquired von Willebrand's syndrome in the course of Waldenstrom disease. Thromb Haemost 1980; 44: 115–118
   PubMed Web of Science ®Google Scholar
• Brody J I, Haidar M E, Rossman R E. A haemorrhagic syndrome in Waldenstrom macroglobulin-aemia secondary to immunoadsorption of factor VIII. N Engl J Med 1979; 300: 408–410
   PubMed Web of Science ®Google Scholar
• Wautier J L, Levy-Toledano S, Caen J P. Acquired von Willebrand's syndrome and thrombopathy in patient with chronic lymphocytic leukemia. Scand J Haematol 1976; 16: 128–134
   PubMedGoogle Scholar
• Roussi J H, Houbouyan L L, Alterescu L, Franc B, Goguel A F. Acquired von Willebrand's syndrome associated with hairy cell leukemia. Br J Haematol 1980; 46: 503–506
   PubMed Web of Science ®Google Scholar
• Rao K P, Kizer J, Jones T J, Anunciado A, Pepkowitz S H, Lazarchick J. Acquired von Willebrand's syndrome associated with an extranodal pulmonary lymphoma. Arch Pathol Lab Med 1988; 112: 47–50
   PubMed Web of Science ®Google Scholar
• Tran-Thang C, Mannucci P M, Schneider P, Federici A, 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
   PubMed Web of Science ®Google Scholar
• Tefferi A, Hanson C A, Kurtin P J, Katzmann J A, Dalton R J, Nichols W L. Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells. Br J Haematol 1997; 96: 850–853
   PubMed Web of Science ®Google Scholar
• Kaywin P, McDonough M, Inact P A, Shattil S J. Platelet function in essential thrombocythemia. N Engl J Med 1978; 299: 505–509
   PubMed Web of Science ®Google Scholar
• Raman B KS, Sawdyk M, Saced S M. Essential thrombocythemia with acquired von Willebrand's disease. Am J Clin Pathol 1987; 88: 102–106
   PubMed Web of Science ®Google Scholar
• van Genderen P JJ, Michiels J J. Primary thrombocythemia: diagnosis, clinical manifestations and management. Ann Hematol 1993; 67: 57–62
   PubMed Web of Science ®Google Scholar
• Budde U, Scharf R E, Franke P, Hartmann-Budde K, Dent J, Ruggeri Z M. Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood 1993; 82: 1749–1757
   PubMed Web of Science ®Google Scholar
• van Genderen P J, Michiels J J, van der Poel-van de Luytgaarde S C, van Vliet H H. Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia: relationship with platelet count. Ann Hematol 1994; 69: 81–84
   PubMed Web of Science ®Google Scholar
• Castaman G, Lattuada A, Ruggeri M, Tosetto A, Mannucci P M, Rodeghiero F. Platelet von Willebrand factor abnormalities in myeloproliferative syndromes. Am J Hematol 1995; 49: 289–293
   PubMed Web of Science ®Google Scholar
• Budde U, van Genderen P JJ. Acquired von Willebrand disease in patients with high platelet counts. Semin Thromb Hemost 1997; 23: 425–431
   PubMed Web of Science ®Google Scholar
• van Genderen P J, Leenknegt H, Michiels J J. The paradox of bleeding and thrombosis in thrombocythemia: is von Willebrand factor the link?. Semin Thromb Hemost 1997; 23: 385–389
   PubMed Web of Science ®Google Scholar
• van Genderen P J, Prins F J, Lucas I S, van de Moesdijk D, van Vliet H H, van Strik R, Michiels J J. Decreased half life time of plasma von Willebrand factor collagen binding activity in essential thrombocythaemia: normalization after cytoreduction of the increased platelet count. Br J Haematol 1997; 99: 832–836
   PubMed Web of Science ®Google Scholar
• van Genderen P J, van Vliet H H, Prins F J, van de Moesdijk D, van Strik R, Zijlstra F J, Budde U, Michiels J J. Excessive prolongation of the bleeding time by aspirin in essential thrombocythemia is related to a decrease of large von Willebrand factor multimers in plasma. Ann Haematol 1997; 75: 215–220
   PubMed Web of Science ®Google Scholar
• Baud P, Tobler A, Lammle B, Alberio L. Acquired von Willebrand syndrome in myeloproliferative disorder. Hamostaseologie 2003; 23: 121–124
   PubMedGoogle Scholar
• Mohri H. Acquired von Willebrand's disease in patients with polycythemia rubra vera. Am J Hematol 1987; 26: 135–146
   PubMed Web of Science ®Google Scholar
• Duran-Saurez J R, Pico M, Zuazu J. Acquired von Willebrand's disease caused by a chronic granulocytic leukaemia. Br J Haematol 1981; 48: 173–174
   PubMedGoogle Scholar
• Mohri H. Acquired von Willebrand's disease and storage pool disease in chronic myelocytic leukemia. Am J Hematol 1986; 22: 391–401
   PubMed Web of Science ®Google Scholar
• Mohri H, Tanabe J, Yamazaki E, Yoshida M, Harano H, Matsuzaki M, Motomura S, Okubo T. Acquired type 2A von Willebrand disease in chronic myelocytic leukemia. Hematopathol Mol Hematol 1996; 10: 123–133
   PubMedGoogle Scholar
• Noronha P A, Houby M A, Maurer H S. Acquired von Willebrand disease in a patient with Wilms' tumor. J Pediatr 1979; 95: 997–999
   PubMed Web of Science ®Google Scholar
• Scott J P, Montgomery R R, Tubergen D G, Hays T. Acquired von Willebrand's disease in association with Wilm's tumor: regression after treatment. Blood 1981; 58: 665–669
   PubMed Web of Science ®Google Scholar
• Bracey A W, Wu A H, Aceves J, Chow T, Carlile S, Hoots W K. Platelet dysfunction associated with Wilms tumor and hyaluronic acid. Am J Hematol 1987; 24: 247–257
   PubMed Web of Science ®Google Scholar
• Coppes M J, Zandroort S WH, Sparling C R, Poon A O, Weitzman S, Blanchette V S. Acquired von Willebrand's disease in Wilms' tumor patient. J Clin Oncol 1992; 10: 422–427
   PubMed Web of Science ®Google Scholar
• Jonge Poerink-Stockschlader A B, Dekker I, Risseeuw-Appel I M, Hahlen K. Acquired von Willebrand's disease in children with Wilms tumor. Med Ped Oncol 1996; 26: 238–243
   PubMedGoogle Scholar
• Leung R S, Liesner R, Brock P. Coagulopathy as a presenting feature of Wilms tumour. Eur J Pediatr 2004; 163: 369–373
   PubMed Web of Science ®Google Scholar
• Facon T, Caron C, Courtin P, Wurtz A, Deghaye M, Bauters F, Mazurier C, Goudemand J. Acquired type II von Willebrand disease associated with adrenal cortical carcinoma. Br J Haematol 1992; 80: 488–494
   PubMed Web of Science ®Google Scholar
• Nowak-Göttl U, Kehrel B, Budde U, Hoffman C, Winkelman W, Jürgens H. Acquired von Willebrand's disease in malignant peripheral neuroectodermal tumor. Med Ped Oncol 1995; 25: 117–118
   PubMedGoogle Scholar
• Soff G A, Green D. Autoantibody to von Willebrand factor in systemic lupus erythematosus. J Lab Clin Med 1992; 121: 424–430
   Web of Science ®Google Scholar
• Niiya M, Niiya K, Takazawa Y, Hayashi Y, Tanio Y, Kushiro M, Tanimizu M, Hasegawa H, Tanimoto M. Acquired type 3-like von Willebrand syndrome preceded full-blown systemic lupus erythematosus. Blood Coagul Fibrinolysis 2002; 13: 361–365
   PubMed Web of Science ®Google Scholar
• Dalton R G, Dewar M S, Savidge G F, Kernoff P B, Matthews K B, Greaves M, Preston F E. Hypothyroidism as a cause of acquired von Willebrand's disease. Lancet 1987; 1: 1007–1009
   PubMed Web of Science ®Google Scholar
• MacCallum P K, Rodgers M, Taberner D A. Hypothyroidism and von Willebrand's disease. Lancet 1987; 1: 1314
   PubMedGoogle Scholar
• Smith S R, Auger M J. Hypothyroidism and von Willebrand's disease. Lancet 1987; 1: 1314
   PubMedGoogle Scholar
• Thorton J G, Paapia L A, Minford A MB. Hypothyroidism and von Willebrand's disease. Lancet 1987; 1: 1314–1315
   PubMedGoogle Scholar
• Palareti G, Biagi G, Legrani C, Bianchi D, Serra D, Savini R, Coccheri S. Association of reduced factor VIII with impaired platelet reactivity to adrenalin and collagen after total thyroidectomy. Thromb Haemost 1989; 62: 1053–1056
   PubMed Web of Science ®Google Scholar
• Blesing N E, Hambley H, McDonald G A. Acquired von Willebrand's disease and hypothyroidism: report of a case presenting with menorrhagia. Postgrad Med J 1990; 66: 474–476
   PubMed Web of Science ®Google Scholar
• Coccia M R, Barnes H V. Hypothyroidism and acquired von Willebrand's disease. J Adolesc Health 1991; 12: 152–154
   PubMed Web of Science ®Google Scholar
• Levesque H, Borg J Y, Cailleux N, Vasse M, Daliphard S, Gancel A, Monconduit M, Courtois H. Acquired von Willebrand's syndrome associated with decrease of plasminogen activator and its inhibitor during hypothyroidism. Eur J Med 1993; 2: 287–288
   PubMedGoogle Scholar
• Bruggers C S, McElligott K, Rallison M L. Acquired von Willebrand in twins with autoimmune hypothyroidism: response to desmopressin and L-thyroxine therapy. J Pediatr 1994; 125: 911–913
   PubMed Web of Science ®Google Scholar
• Tjan-Heijen V C, Harthoom-Lasthuizen K J, Kurstjens R M, Koolen M I. A patient with postpartum primary hypothyroidism and acquired von Willebrand's disease. Neth J Med 1994; 44: 91–94
   PubMed Web of Science ®Google Scholar
• Attivissimo L A, Lichtman S M, Klein I. Acquired von Willebrand syndrome causing a hemorrhagic diathesis in a patient with hypothyroidism. Thyroid 1995; 5: 399–401
   PubMed Web of Science ®Google Scholar
• Aylesworth C A, Smallridge R C, Rick M E, Alving B M. Acquired von Willebrand's disease: a rare manifestation of postpartum thyroiditis. Am J Hematol 1995; 50: 217–219
   PubMed Web of Science ®Google Scholar
• Franchini M, Zugni C, Veneri D, Gandini G, Lippi G, Manzato F, Brazzarola P. High prevalence of acquired von Willebrand's syndrome in patients with thyroid diseases undergoing thyroid surgery. Haematologica 2004; 89: 1341–1346
   PubMed Web of Science ®Google Scholar
• Franchini M, Veneri D, Lippi G. Analysis of thyroid hormone status in 131 consecutive individuals with low von Willebrand factor levels. Thromb Haemost 2005; 93: 392–393
   PubMed Web of Science ®Google Scholar
• Leone G, Pola P, Guerrera G, Bizzi B. Acquired von Willebrand's syndrome during collagen disorders. Haematologica 1974; 59: 212–220
   PubMedGoogle Scholar
• Mannucci P M, Lombardi R, Lattuada A, Perticucci E, Valsecchi R, Remuzzi G. Supranormal von Willebrand factor multimers in scleroderma. Blood 1987; 73: 1586–1591
   Web of Science ®Google Scholar
• Yoshida H, Arai K, Wakashin M. Development of acquired von Willebrand's disease after mixed connective tissue disease. Am J Med 1988; 85: 445–446
   PubMed Web of Science ®Google Scholar
• Lazarchick J, Green C. Acquired von Willebrand disease following bone marrow transplantation. Ann Clin Lab Sci 1994; 24: 211–215
   PubMed Web of Science ®Google Scholar
• Gill J C, Wilson A D, Endres-Brooks J, Montgomery R R. Loss of the largest von Willebrand factor multimers from the plasma of patients with congenital cardiac defects. Blood 1986; 67: 758–761
   PubMed Web of Science ®Google Scholar
• Rauch R, Budde U, Girish M, Klinge J, Hofbeck M. Acquired von Willebrand disease in an infant. Resolution by interventional occlusion of patent ductus arteriosus. Thromb Res 2001; 102: 407–409
   PubMed Web of Science ®Google Scholar
• Froom P, Margulis T, Grenadier E, Palant A, David M, Aghai E. von Willebrand factor and mitral valve prolapse. Thromb Haemost 1988; 60: 230–231
   PubMed Web of Science ®Google Scholar
• Warkentin T E, Moore J C, Morgan D G. Aortic stenosis and bleeding gastrointestinal angiodysplasia: is acquired von Willebrand's disease the link?. Lancet 1992; 340: 35–37
   PubMed Web of Science ®Google Scholar
• O'Brien J R, Etherington M D. Heart valve stenosis and von Willebrand's factor multimers. Lancet 1992; 340: 616
   PubMed Web of Science ®Google Scholar
• Anderson R P, McGrath K, Street A. Reversal of aortic stenosis, bleeding gastrointestinal angiodysplasia, and von Willebrand syndrome by aortic valve replacement. Lancet 1996; 347: 689–690
   PubMed Web of Science ®Google Scholar
• 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: 343–349
   PubMed Web of Science ®Google Scholar
• 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: 490–494
   PubMed Web of Science ®Google Scholar
• Conrad M E, LaTour L F. Acquired von Willebrand's disease, IgE polyclonal gammopathy and griseofulvin therapy. Am J Hematol 1992; 41: 143
   Web of Science ®Google Scholar
• Castaman G, Rodeghiero F. Acquired transitory von Willebrand syndrome with ciprofloxacin. Lancet 1994; 343: 492
   PubMed Web of Science ®Google Scholar
• Castaman G, Lattuada A, Mannucci P M, Rodeghiero F. Characterization of two cases of acquired transitory von Willebrand syndrome with ciprofloxacin. Am J Hematol 1995; 49: 83–86
   PubMed Web of Science ®Google Scholar
• Kreuz W, Linde R, Funk M, Meyer-Schrod R, Meyer-Schrod R, Foll E, Nowak-Gottl U, Jacobi G, Vigh Z, Scharrer I. Induction of von Willebrand disease type I by valproic acid. Lancet 1990; 335: 1350–1351
   PubMed Web of Science ®Google Scholar
• Strauss R G, Stump D C, Henriksen R A. Hydroxyethyl starch accentuates von Willebrand's disease. Transfusion 1985; 25: 235–237
   PubMed Web of Science ®Google Scholar
• Sanfelippo M J, Subernola P D, Geimer N F. Development of a von Willebrand-like syndrome after use of hydroxyl starch. Am J Clin Pathol 1987; 88: 653–655
   PubMedGoogle Scholar
• Dalrymphyle-Hay M, Aitchison R, Collins R, Sekhar M, Calvin B. Hydroxy-ethyl starch induced acquired von Willebrand's disease. Clin Lab Haematol 1992; 14: 209–211
   PubMedGoogle Scholar
• Rock G, Adamkiewicz T, Blanchette V, Poon A, Sparling C. Acquired von Willebrand factor deficiency during high-dose infusion of recombinant factor VIII. Br J Haematol 1996; 93: 684–687
   PubMed Web of Science ®Google Scholar
• Gralnick H R, McKeown L P, Williams S B, Strafer B C, Pierce L. Plasma and platelet von Willebrand factor defects in uremia. Am J Med 1988; 85: 806–810
   PubMed Web of Science ®Google Scholar
• Rodeghiero F, Castaman G, Lombardi R, Mannucci P M. von Willebrand factor abnormalities in two patients with uraemia. Lancet 1988; 1: 1016–1017
   Google Scholar
• Castaman G C, Rodeghiero F, Lattuada A, La Greca C Z, Mannucci P M. Multimeric pattern of plasma and platelet von Willebrand factor is normal in uremic patients. Am J Hematol 1993; 44: 266–269
   PubMed Web of Science ®Google Scholar
• Wautier J L, Caen J P, Rymer R. Angiodysplasia in acquired von Willebrand disease. Lancet 1976; 2: 973
   PubMed Web of Science ®Google Scholar
• Rosborough T K, Swaim W R. Acquired von Willebrand's disease, platelet-release defect and angiodysplasia. Am J Med 1978; 65: 96–100
   PubMed Web of Science ®Google Scholar
• McGrath K M, Johnson C A, Stuart J J. Acquired von Willebrand disease associated with an inhibitor to factor VIII antigen and gastrointestinal telangiectasia. Am J Med 1979; 67: 693–696
   PubMedGoogle Scholar
• Cass A J, Bliss B P, Bolton R P, Cooper B T. Gastrointestinal bleeding, angiodysplasia of the colon and acquired von Willebrand's disease. Br J Surg 1980; 67: 639–641
   PubMed Web of Science ®Google Scholar
• Woodlock T J, Francis C W, Rowe J M, Brown M J, Marder V J. Prolonged remission after life-threatening gastrointestinal hemorrhage from coexistent angiodysplasia and acquired bleeding diathesis. Am J Hematol 1988; 27: 125–131
   PubMed Web of Science ®Google Scholar
• Fressinaud E, Meyer D. International survey of patients with von Willebrand disease and angiodysplasia. Thromb Haemost 1993; 70: 546
   PubMed Web of Science ®Google Scholar
• Inbal A, Bank I, Zivelin A, Varon D, Dardik R, Shapiro R, Rosenthal E, Shenkman B. Acquired von Willebrand disease in a patient with angiodysplasia resulting from immune-mediated clearance of von Willebrand factor. Br J Haematol 1997; 96: 179–182
   PubMed Web of Science ®Google Scholar
• Alhumood S A, Devine D V, Lawson L, Nantel S H, Carter C J. Idiopathic immune-mediated acquired von Willebrand's disease in a patient with angiodysplasia: demonstration of an unusual inhibitor causing a functional defect and rapid clearance of von Willebrand factor. Am J Hematol 1999; 60: 151–157
   PubMed Web of Science ®Google Scholar
• Warkentin T E, Moore C J, Anand S S, Lonn E M, Morgan D J. Gastrointestinal bleeding, angiodysplasia, cardiovascular disease, and acquired von Willebrand syndrome. Transf Med Rev 2003; 17: 272–286
   PubMed Web of Science ®Google Scholar
• Kinoshita S, Yoshioka K, Kasahara M, Takamiya O. Acquired von Willebrand disease after Epstein-Barr virus infection. J Pediatrics 1991; 119: 595–598
   PubMed Web of Science ®Google Scholar
• Richard C, Sedano M C, Cuadrado M A, 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
   PubMed Web of Science ®Google Scholar
• Clough V, MacFarlane I A, O'Connor J, Woud J K. Acquired von Willebrand's syndrome and Ehlers-Danlos syndrome presenting with gastrointestinal bleeding. Scand J Haematol 1979; 22: 305–310
   PubMedGoogle Scholar
• Parker R I, McKeown L P, Grallin S I, Gralnick H R. Absence of the largest platelet von Willebrand multimers in a patient with lactoferrin deficiency and a bleeding tendency. Thromb Haemost 1992; 67: 320–324
   PubMed Web of Science ®Google Scholar
• Benson P J, Peterson L C, Hasegaura D K, Smith C M. Abnormality of von Willebrand factor in patients with hemoglobin E-β thalassemia. Am J Clin Pathol 1990; 93: 395–399
   PubMed Web of Science ®Google Scholar
• Sokol L, Stueben E T, Jaikishen J P, Lamarche M B. Turner syndrome associated with acquired von Willebrand disease, primary biliary cirrhosis, and inflammatory bowel disease. Am J Hematol 2002; 70: 257–259
   PubMed Web of Science ®Google Scholar
• Stableforth P, Tamagnini G L, Dormandy K M. Acquired von Willebrand syndrome with inhibitors both to factor VIII clotting activity and ristocetin-induced platelet aggregation. Br J Haematol 1976; 33: 565–573
   PubMed Web of Science ®Google Scholar
• Franchini M, De Gironcoli M, Lippi G, Manzato F, Brazzarola P, Bottura D, Aprili G, Gandini G. Efficacy of desmopressin as surgical prophylaxis in patients with acquired von Willebrand disease undergoing thyroid surgery. Haemophilia 2002; 8: 142–144
   PubMed Web of Science ®Google Scholar
• Kumar S, Pruthi R K, Nichols W L. Acquired von Willebrand disease. Mayo Clinic Proc 2002; 77: 181–187
   PubMed Web of Science ®Google Scholar
• van Genderen P J, Vink T, Michiels J J, van't Veer M B, Sixma J J, van Vliet H H. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 1994; 84: 3378–3384
   PubMed Web of Science ®Google Scholar
• Goudemand J, Samor B, Caron C, Jude B, Gosset D, Mazurier C. Acquired type II von Willebrand's disease: demonstration of a complexed inhibitor of the von Willebrand factor-platelet interaction and response to treatment. Br J Haematol 1988; 68: 227–233
   PubMed Web of Science ®Google Scholar
• Fricke W A, Brinkhous K M, Garris J B, Roberts H R. Comparison of inhibitory and binding characteristics of an antibody causing acquired von Willebrand syndrome: an assay for von Willebrand factor binding by antibody. Blood 1985; 66: 562–569
   PubMed Web of Science ®Google Scholar
• Mohri H, Motomura S, Kanamori H, Matsuzaki M, Watanabe S, Maruta A, Kodama F, Okubo T. Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood 1998; 91: 3623–3629
   PubMed Web of Science ®Google Scholar
• Gan T E, Sawers R J, Koutts J. Pathogenesis of antibody-induced acquired von Willebrand syndrome. Am J Hematol 1980; 9: 363–371
   PubMed Web of Science ®Google Scholar
• Scrobohaci M L, Daniel M T, Levy Y, Marolleau J P, Brouet J C. Expression of GpIb on plasma cells in a patient with monoclonal IgG and acquired von Willebrand disease. Br J Haematol 1993; 84: 471–475
   PubMed Web of Science ®Google Scholar
• Pareti F I, Lattuada A, Bressi C, Zanobini M, Sala A, Steffan A, Ruggeri Z M. Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis. Circulation 2000; 102: 1290–1295
   PubMed Web of Science ®Google Scholar
• Sadler J E. Aortic stenosis, von Willebrand factor and bleeding. N Engl J Med 2003; 349: 323–325
   PubMed Web of Science ®Google Scholar
• Favaloro E J, Facey D, Grispo L. Laboratory assessment of von Willebrand factor. Use of different assays can influence the diagnosis of von Willebrand's disease, dependent on differing sensitivity of sample preparation and differential recognition of high molecular weight VWF forms. Am J Clin Pathol 1995; 104: 264–271
   PubMed Web of Science ®Google Scholar
• van Genderen P J, Vink T, Michiels J J, Van't Veer M B, Sixma J J, van Vliet H H. Acquired von Willebrand disease caused by an antibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 1994; 84: 3378–3384
   PubMed Web of Science ®Google Scholar
• Siaka C, Rugeri L, Caron C, Goudemand J. A new ELISA assay for diagnosis of acquired von Willebrand syndrome. Haemophilia 2003; 9: 303–308
   PubMed Web of Science ®Google Scholar
• McCarroll D R, Ruggeri Z M, Montgomery R R. Correlation between circulating levels of von Willebrand's antigen II and von Willebrand factor: discrimination between type I and type II von Willebrand's disease. J Lab Clin Med 1984; 103: 704–711
   PubMed Web of Science ®Google Scholar
• Genderen P JJ, Boertjes R C, Mourik J A. Quantitative analysis of von Willebrand factor and its propeptide in plasma in acquired von Willebrand syndrome. Thromb Haemost 1998; 80: 495–498
   PubMed Web of Science ®Google Scholar
• Friederich P W, Wever P C, Briet E, Doorenbos C J, 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
   PubMed Web of Science ®Google Scholar
• Smaradottir A, Bona R. A case of acquired von Willebrand syndrome successfully treated with recombinant factor VIIa during thyroidectomy. Thromb Haemost 2005; 93: 1009
   Web of Science ®Google Scholar
• Federici A B. Use of intravenous immunoglobulin in patients with acquired von Willebrand syndrome. Hum Immunol 2005; 66: 422–430
   PubMed Web of Science ®Google Scholar
• 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: 33–39
   PubMedGoogle Scholar
• Michiels J J, van Vliet H H. Willebrand disease in monoclonal gammopathies: effectiveness of high-dose intravenous immunoglobulin. Clin Appl Thromb Hemost 1999; 5: 152–157
   PubMedGoogle Scholar
• Van Genderen P J, Michiels J J, Bakker J J, van't Veer M B. Effectiveness of high-dose gamma globulin therapy in acquired von Willebrand's disease. Vox Sang 1994; 67: 14–17
   PubMed Web of Science ®Google Scholar
• Castaman G, Tosetto A, Rodeghiero F. Effectiveness of high-dose intravenous immunoglobulin in a case of acquired von Willebrand syndrome with chronic melena not responsive to desmopressin and factor VII concentrate. Am J Hematol 1992; 41: 132–136
   PubMed Web of Science ®Google Scholar
• Delannoy A, Saillez A C. High-dose intravenous gammaglobulin for acquired von Willebrand's disease. Br J Haematol 1988; 70: 387
   PubMed Web of Science ®Google Scholar
• Arkel Y S, Lynch J, Kamiyama M. Treatment of acquired von Willebrand syndrome with intravenous immunoglobulin. Thromb Haemost 1994; 72: 643–644
   PubMed Web of Science ®Google Scholar
• Hanley D, Arkel Y S, Lynch J, Kamiyama M. Willebrand's syndrome in association with a lupus-like anticoagulant corrected by intravenous immunoglobulin. Am J Hematol 1994; 46: 141–146
   PubMed Web of Science ®Google Scholar
• Delmer A, Horellou M H, Brechot J M, Prudent J, Potevin F, Lecrubier C, Girard-Longhini C, Samama M, Zittoun R. Acquired von Willebrand disease: correction of hemostatic defect by high-dose intravenous immunoglobulins. Am J Hematol 1992; 40: 151–152
   PubMed Web of Science ®Google Scholar
• Macik B G, Gabriel D A, White G C, II, High K, Roberts H. The use of high-dose intravenous gamma-globulin in acquired von Willebrand syndrome. Arch Pathol Lab Med 1988; 112: 143–146
   PubMed Web of Science ®Google Scholar
• Lipkind H S, Kurtis J D, Powrie R, Carpenter M W. Acquired von Willebrand disease: management of labor and delivery with intravenous dexamethasone, continuous factor concentrate, and immunoglobulin infusion. Am J Obstet Gynecol 2005; 192: 2067–2070
   PubMed Web of Science ®Google Scholar
• Frank R D, Kunz D, Wirtz D C. 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
   PubMed Web of Science ®Google Scholar
• Silberstein L E, Abrahm J, Shattil S J. The efficacy of intensive plasma exchange in acquired von Willebrand's disease. Transfusion 1987; 27: 234–237
   PubMed Web of Science ®Google Scholar
• Clough V, MacFarlane I A, O'Connor J, Wood J K. Acquired von Willebrand's syndrome and Ehlers-Danlos syndrome presenting with gastro-intestinal bleeding. Scand J Haematol 1979; 22: 305–310
   PubMedGoogle Scholar
• Joist J H, Cowan F, Zimmerman T S. Acquired von Willebrand's disease. Evidence for a quantitative and qualitative factor VIII disorder. N Engl J Med 1978; 298: 988–991
   PubMed Web of Science ®Google Scholar
• Cattaneo M. Role of von Willebrand factor in atherothrombosis. Haematologica 2001; 86: 3–5
   PubMedGoogle Scholar
• Yamashita A, Asada Y, Sugimura H, Yamamoto H, Marutsuka K, Hatakeyama K, Tamura S, Ikeda Y, Sumiyoshi A. Contribution of von Willebrand factor to thrombus formation on neointima of rabbit stenotic iliac artery under high blood-flow velocity. Arterioscler Thromb Vasc Biol 2003; 23: 1105–1110
   PubMed Web of Science ®Google Scholar
• Gill J C, Endres-Brooks J, Bauer P J, Marks W J, Jr, Montgomery R R. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987; 69: 1691–1695
   PubMed Web of Science ®Google Scholar
• Jeremic M, Weisert O, Gedde-Dahl T W. Factor VIII (AHG) levels in 1016 regular blood donors. The effects of age, sex, and ABO blood groups. Scand J Clin Lab Invest 1976; 36: 461–466
   PubMed Web of Science ®Google Scholar
• Mannucci P M. von Willebrand factor: a marker of endothelial damage?. Arterioscler Thromb Vasc Biol 1998; 18: 1359–1362
   PubMed Web of Science ®Google Scholar
• Meade T W, Cooper J A, Stirling Y, Howarth D J, Ruddock V, Miller G J. Factor VIII, ABO blood group and the incidence of ischaemic heart disease. Br J Haematol 1994; 88: 601–607
   PubMed Web of Science ®Google Scholar
• Thompson S G, Kienast J, Pyke S D, Haverkate F, van de Loo J C. Hemostatic factors and the risk of myocardial infarction or sudden death in patients with angina pectoris. European Concerted Action on Thrombosis and Disabilities Angina Pectoris Study Group. N Engl J Med 1995; 332: 635–641
   PubMed Web of Science ®Google Scholar
• Folsom A R, Wu K K, Rosamond W D, Sharrett A R, Chambless L E. Prospective study of hemostatic factors and incidence of coronary heart disease: the Atherosclerosis Risk in Communities (ARIC) Study. Circulation 1997; 96: 1102–1108
   PubMed Web of Science ®Google Scholar
• Folsom A R, Rosamond W D, Shahar E, Cooper L S, Aleksic N, Nieto F J, Rasmussen M L, Wu K K. Prospective study of markers of hemostatic function with risk of ischemic stroke. The Atherosclerosis Risk in Communities (ARIC) Study Investigators. Circulation 1999; 100: 736–742
   PubMed Web of Science ®Google Scholar
• Smith F B, Lee A J, Fowkes F G, Price J F, Rumley A, Lowe G D. Hemostatic factors as predictors of ischemic heart disease and stroke in the Edinburgh Artery Study. Arterioscler Thromb Vasc Biol 1997; 17: 3321–3325
   PubMed Web of Science ®Google Scholar
• Rumley A, Lowe G D, Sweetnam P M, Yarnell J W, Ford R P. Factor VIII, von Willebrand factor and the risk of major ischaemic heart disease in the Caerphilly Heart Study. Br J Haematol 1995; 105: 110–116
   Google Scholar
• Jansson J H, Nilsson T K, Johnson O. von Willebrand factor, tissue plasminogen activator, and dehydroepiandrosterone sulphate predict cardiovascular death in a 10 year follow up of survivors of acute myocardial infarction. Heart 1998; 80: 334–337
   PubMed Web of Science ®Google Scholar
• Whincup P H, Danesh J, Walker M, Lennon L, Thomson A, Appleby P, Rumley A, Lowe G D. von Willebrand factor and coronary heart disease: prospective study and meta-analysis. Eur Heart J 2002; 23: 1764–1770
   PubMed Web of Science ®Google Scholar
• Jager A, van Hinsbergh V W, Kostense P J, Emeis J J, Yudkin J S, Nijpels G, Dekker J M, Heine R J, Bouter L M, Stehouwer C D. von Willebrand factor, C-reactive protein, and 5-year mortality in diabetic and nondiabetic subjects: the Hoorn Study. Arterioscler Thromb Vasc Biol 1999; 19: 3071–3078
   PubMed Web of Science ®Google Scholar
• Morange P E, Simon C, Alessi M C, Luc G, Arveiler D, Ferrieres J, Amouyel P, Evans A, Ducimetiere P, Juhan-Vague I. PRIME Study Group. Endothelial cell markers and the risk of coronary heart disease: the Prospective Epidemiological Study of Myocardial Infarction (PRIME) study. Circulation 2004; 109: 1343–1348
   PubMed Web of Science ®Google Scholar
• van der Meer I M, Brouwers G J, Bulk S, Leebeek F W, van der Kuip D A, Hofman A, Witteman J C, Gomez Garcia E B. Genetic variability of von Willebrand factor and risk of coronary heart disease: the Rotterdam Study. Br J Haematol 2004; 124: 343–347
   PubMed Web of Science ®Google Scholar
• Conway D S, Pearce L A, Chin B S, Hart R G, Lip G Y. Prognostic value of plasma von Willebrand factor and soluble P-selectin as indices of endothelial damage and platelet activation in 994 patients with nonvalvular atrial fibrillation. Circulation 2003; 107: 3141–3145
   PubMed Web of Science ®Google Scholar
• Sramek A, Bucciarelli P, Federici A B, Mannucci P M, De Rosa V, Castaman G, Morfini M, Mazzucconi M G, Rocino A, Schiavoni M, Scaraggi F A, Reiber J H, Rosendaal F R. Patients with type 3 severe von Willebrand disease are not protected against atherosclerosis: results from a multicenter study in 47 patients. Circulation 2004; 109: 740–744
   PubMed Web of Science ®Google Scholar
• Stead N W, Bauer K A, Kinney T R, Lewis J G, Campbell E E, Shifman M A, Rosenberg R D, Pizzo S V. Venous thrombosis in a family with defective release of vascular plasminogen activator and elevated plasma factor VIII/von Willebrand's factor. Am J Med 1983; 74: 33–39
   PubMed Web of Science ®Google Scholar
• Nilsson T, Mellbring G, Hedner U. Relationship between factor XII, von Willebrand factor and postoperative deep vein thrombosis. Acta Chir Scand 1986; 152: 347–349
   PubMedGoogle Scholar
• Koster T, Blann A D, Briet E, Vandenbroucke J P, Rosendaal F R. Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet 1995; 345: 152–155
   PubMed Web of Science ®Google Scholar
• Tsai A W, Cushman M, Rosamond W D, Heckbert S R, Tracy R P, Aleksic N, Folsom A R. Coagulation factors, inflammation markers, and venous thromboembolism: the longitudinal investigation of thromboembolism etiology (LITE). Am J Med 2002; 113: 636–642
   PubMed Web of Science ®Google Scholar
• Bucek R A, Reiter M, Quehenberger P, Weltermann A, Kyrle P A, Minar E. Thrombus precursor protein, endogenous thrombin potential, von-Willebrand factor and activated factor VII in suspected deep vein thrombosis: is there a place for new parameters?. Br J Haematol 2003; 120: 123–128
   PubMed Web of Science ®Google Scholar
• Kamphuisen P W, Eikenboom J C, Rosendaal F R, Koster T, Blann A D, Vos H L, Bertina R M. High factor VIII antigen levels increase the risk of venous thrombosis but are not associated with polymorphisms in the von Willebrand factor and factor VIII gene. Br J Haematol 2001; 115: 156–158
   PubMed Web of Science ®Google Scholar
• Franchini M, Zaffanello M, Veneri D. Advances in the pathogenesis, diagnosis and treatment of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Thromb Res 2006; 118: 177–184
   PubMed Web of Science ®Google Scholar
• Moake J L. Moschcowitz, multimers, and metalloprotease. N Engl J Med 1998; 339: 1629–1631
   PubMed Web of Science ®Google Scholar
• Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 2001; 60: 831–846
   PubMed Web of Science ®Google Scholar
• Tsai H M. Deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura. Arterioscler Thromb Vascular Biol 2003; 23: 388–396
   PubMed Web of Science ®Google Scholar
• Moake J L, Rudy C K, Troll J H, Weinstein M J, Colannino N M, Azocar J, Seder R H, Hong S L, Deykin D. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 1982; 307: 1432–1435
   PubMed Web of Science ®Google Scholar
• Furlan M, Robles R, Lammle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996; 87: 4223–4234
   PubMed Web of Science ®Google Scholar
• Tsai H M. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87: 4235–4244
   PubMed Web of Science ®Google Scholar
• Furlan M, Robles R, Solenthaler M, Lammle B. Acquired deficiency of von Willebrand factor cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood 1998; 91: 2839–2846
   PubMed Web of Science ®Google Scholar
• Tsai H M, Lian E C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339: 1585–1594
   PubMed Web of Science ®Google Scholar
• Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle P A, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lammle B. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome. N Engl J Med 1998; 339: 1578–1584
   PubMed Web of Science ®Google Scholar
• Veyradier A, Obert B, Houllier A, Meyer D, Girma J P. Specific von Willebrand factor-cleaving protease in thrombotic microangiopathy: a study of 111 cases. Blood 2001; 98: 1765–1772
   PubMed Web of Science ®Google Scholar
• Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor cleaving protease and its identification as a new member of the metalloproteinase family. Blood 2001; 98: 1662–1666
   PubMed Web of Science ®Google Scholar
• Gerritsen H E, Robles R, Lammle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98: 1654–1661
   PubMed Web of Science ®Google Scholar
• Zheng X, Chung D, Takayama T K, Majerus E M, Sadler J E, Fujikawa K. Structure of von Willebrand factor cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem 2001; 276: 41059–41063
   PubMed Web of Science ®Google Scholar
• Plaimauer B, Zimmermann K, Völkel D, Antoine G, Kerschbaumer R, Jenab P, Furlan M, Gerritsen H, Lammle B, Schwarz H P, Scheiflinger F. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood 2002; 100: 3626–3632
   PubMed Web of Science ®Google Scholar
• Levy G G, Nichols W C, Lian E C, Foroud T, McClintick J N, McGee B M, Yang A Y, Siemieniak D R, Stark K R, Gruppo R, Sarode R, Shurin S B, Chandrasekaran V, Stabler S P, Sabio H, Bouhassira E E, Upshaw J D, Jr, Ginsburg D, Tsai H M. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001; 413: 488–494
   PubMed Web of Science ®Google Scholar
• Amorosi E L, Ultmann J E. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine (Baltimore) 1966; 45: 139–159
   Web of Science ®Google Scholar
• Cuttner J. Thrombotic thrombocytopenic purpura: a ten-year experience. Blood 1980; 56: 302–306
   PubMed Web of Science ®Google Scholar
• Petitt R M. Thrombotic thrombocytopenic purpura: a thirty-year review. Semin Thromb Hemost 1980; 6: 350–355
   PubMed Web of Science ®Google Scholar
• Ridolfi R L, Bell W R. Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature. Medicine (Baltimore) 1981; 60: 413–428
   PubMed Web of Science ®Google Scholar
• Kwaan H C. Clinicopathologic features of thrombotic thrombocytopenic purpura. Semin Hematol 1987; 24: 71–81
   PubMed Web of Science ®Google Scholar
• Murrin R JA, Murray J A. Thrombotic thrombocytopenic purpura: etiology, pathophysiology and treatment. Blood Rev 2006; 29: 51–60
   Google Scholar
• Allford S L, Hunt B J, Rose P, Machin S J. Haemostosis and Thrombosis Task Force, British Committee for Standards in Haematology. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol 2003; 120: 556–573
   PubMed Web of Science ®Google Scholar
• Veyradier A, Meyer D. Thombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost 2005; 3: 2420–2427
   PubMed Web of Science ®Google Scholar
• Bell W R, Braine H G, Ness P M, Kickler T S. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991; 325: 398–403
   PubMed Web of Science ®Google Scholar
• Rock G A, Shumak K H, Buskard N A, Blanchette V S, Kelton J G, Nair R C, Spasoff R A. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 1991; 325: 393–397
   PubMed Web of Science ®Google Scholar
• Nabhan C, Kwaan H C. Current concepts in the diagnosis and management of thrombotic thrombocytopenic purpura. Hematol Oncol Clin North Am 2003; 17: 177–199
   PubMed Web of Science ®Google Scholar
• Moake J L. Thrombotic microangiopathies. N Engl J Med 2002; 347: 589–600
   PubMed Web of Science ®Google Scholar
• Kwaan H C, Soff G A. Management of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Semin Hematol 1997; 34: 159–166
   PubMed Web of Science ®Google Scholar
• Shepard K V, Bukowski R M. The treatment of thrombotic thrombocytopenic purpura with exchange transfusions, plasma infusions, and plasma exchange. Semin Hematol 1987; 24: 178–193
   PubMed Web of Science ®Google Scholar
• Hayward C P, Sutton D M, Carter W H, Jr, Campbell E D, Scott J G, Francombe W H, Shumak K H, Baker M A. Treatment outcomes in patients with adult thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Arch Intern Med 1994; 154: 982–987
   PubMed Web of Science ®Google Scholar
• Pisciotta A V, Garthwaite T, Darin J, Aster R H. Treatment of thrombotic thrombocytopenic purpura by exchange transfusion. Am J Hematol 1977; 3: 73–82
   PubMed Web of Science ®Google Scholar
• Rock G A. Management of thrombotic thrombocytopenic purpura. Br J Haematol 2000; 109: 496–507
   PubMed Web of Science ®Google Scholar
• Rock G, Shumak K H, Sutton D M, Buscard N A, Nair R C. The Members of the Canadian Apheresis Group. Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura. Br J Haem 1996; 94: 383–386
   PubMed Web of Science ®Google Scholar
• Zeigler Z, Shadduck R K, Gryn J F, Rintels P B, George J N, Besa E C, Bodensteiner D, Silver B, Kramer R E, North American TTP Group. Cryoprecipitate poor plasma does not improve early response in primary adult thrombotic thrombocytopenic purpura (TTP). J Clin Apheresis 2001; 16: 19–22
   PubMed Web of Science ®Google Scholar
• Raife T J, Friedman K D, Dwyre D M. The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma. Transfusion 2006; 46: 74–79
   PubMed Web of Science ®Google Scholar
• Sadler J E, Moake J L, Miyata T, George J N. Recent advances in thrombotic thrombocytopenic purpura. Hematology (Am Soc Hematol Educ Program) 2004; 407–423
   PubMedGoogle Scholar
• del a Rubia J, Lopez A, Arriaga F, Cid A R, Vicente A I, Marty M L, Sanz M A. Response to plasma exchange and steroids as combined therapy for patients with thrombotic thrombocytopenic purpura. Acta Haematol 1999; 102: 12–16
   PubMed Web of Science ®Google Scholar
• Yomtovian R, Niklinski W, Silver B, Sarode R, Tsai H M. Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature. Br J Haematol 2004; 124: 787–795
   PubMed Web of Science ®Google Scholar
• Ahmad A, Aggarwal A, Sharma D, Dave H P, Kinsella V, Rick M E, Schechter G P. Rituximab for treatment of refractory/relapsing thrombotic thrombocytopenic purpura (TTP). Am J Hematol 2004; 77: 171–176
   PubMed Web of Science ®Google Scholar
• Kremer Hovinga J A, Studt J D, Demarmels Biasiutti F, Solenthaler M, Alberio L, Zwicky C, Fontana S, Taleghani B M, Tobler A, Lammle B. Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura. Haematologica 2004; 89: 320–324
   PubMed Web of Science ®Google Scholar
• Gutterman L A, Stevenson T D. Treatment of thrombotic thrombocytopenic purpura with vincristine. JAMA 1982; 247: 1433–1436
   PubMed Web of Science ®Google Scholar
• Plaimauer B, Scheiflinger F. Expression and characterization of recombinant human ADAMTS-13. Semin Hematol 2004; 41: 24–33
   PubMed Web of Science ®Google Scholar