Accessible Search Form           Advanced Search

Skip left side navigation and go to content

Health Professionals

References

  1. Sadler JE, Mannucci PM, 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 Aug;84(2):160-174.
  2. Biron C, Mahieu B, Rochette A, Capdevila X, Castex A, Amiral J, D'Athis F, Schved JF. Preoperative screening for von Willebrand disease type 1: low yield and limited ability to predict bleeding. J Lab Clin Med 1999 Dec;134(6):605-609.
  3. Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand's disease. Blood 1987 Feb;69(2):454-459.
  4. Werner EJ, Broxson EH, Tucker EL, Giroux DS, Shults J, Abshire TC. Prevalence of von Willebrand disease in children: a multiethnic study. J Pediatr 1993 Dec;123(6):893-898.
  5. Hallberg L, Hogdahl AM, Nilsson L, Rybo G. Menstrual blood loss—a population study. Variation at different ages and attempts to define normality. Acta Obstet Gynecol Scand 1966; 45(3):320-351.
  6. Association of Hemophilia Clinic Directors of Canada. Hemophilia and von Willebrand's disease: 1. Diagnosis, comprehensive care and assessment. Can Med Assoc J 1995 Jul;153(1):19-25.
  7. Federici AB, Castaman G, Mannucci PM, for the Italian Association of Hemophilia Centers (AICE). Guidelines for the diagnosis and management of von Willebrand disease in Italy. Haemophilia 2002 Sep;8(5):607-621.
  8. Laffan M, Brown SA, Collins PW, Cumming AM, Hill FG, Keeling D, Peake IR, Pasi KJ. The diagnosis of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004 May;10(3):199-217.
  9. Pasi KJ, Collins PW, Keeling DM, Brown SA, Cumming AM, Dolan GC, Hay CR, Hill FG, Laffan M, Peake IR. Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization. Haemophilia 2004 May;10(3):218-231.
  10. Von Willebrand EA. Hereditary pseudohaemophilia [English translation]. Haemophilia 1999 May;5(3):223-231.
  11. Montgomery RR, Gill JC. Interactions between von Willebrand factor and factor VIII: where did they first meet. J Pediatr Hematol Oncol 2000 May;22(3):269-275.
  12. Rosenberg JB, Greengard JS, Montgomery RR. Genetic induction of a releasable pool of factor VIII in human endothelial cells. Arterioscler Thromb Vasc Biol 2000 Dec;20(12):2689-2695.
  13. Miller CH, Haff E, Platt SJ, Rawlins P, Drews CD, Dilley AB, Evatt B. Measurement of von Willebrand factor activity: relative effects of ABO blood type and race. J Thromb Haemost 2003 Oct;1(10):2191-2197.
  14. Sukhu K, Poovalingam V, Mahomed R, Giangrande PL. Ethnic variation in von Willebrand factor levels can influence the diagnosis of von Willebrand disease. Clin Lab Haematol 2003 Aug;25(4):247-249.
  15. Ginsburg D, Handin RI, Bonthron DT, Donlon TA, Bruns GA, Latt SA, Orkin SH. Human von Willebrand factor (vWF): isolation of complementary DNA (cDNA) clones and chromosomal localization. Science 1985 Jun;228 (4706):1401-1406.
  16. Mancuso DJ, Tuley EA, Westfield LA, Worrall NK, Shelton-Inloes BB, Sorace JM, Alevy YG, Sadler JE. Structure of the gene for human von Willebrand factor. J Biol Chem 1989 Nov;264(33):19514-19527.
  17. Patracchini P, Calzolari E, Aiello V, Palazzi P, Banin P, Marchetti G, Bernardi F. Sublocalization of von Willebrand factor pseudogene to 22q11.22-q11.23 by in situ hybridization in a 46,X, t(X;22)(pter;q11.21) translocation. Hum Genet 1989 Oct;83(3):264-266.
  18. Mancuso DJ, Tuley EA, Westfield LA, Lester-Mancuso TL, Le Beau MM, Sorace JM, Sadler JE. Human von Willebrand factor gene and pseudogene: structural analysis and differentiation by polymerase chain reaction. Biochemistry 1991 Jan;30(1):253-269.
  19. Sadler JE. von Willebrand factor. J Biol Chem 1991 Dec;266(34):22777-22780.
  20. Eikenboom JC, Vink T, Briet E, Sixma JJ, Reitsma PH. Multiple substitutions in the von Willebrand factor gene that mimic the pseudogene sequence. Proc Natl Acad Sci USA 1994 Mar;91(6):2221-2224.
  21. Eikenboom JC, Castaman G, Vos HL, Bertina RM, Rodeghiero F. Characterization of the genetic defects in type 1 and type 3 von Willebrand disease patients of Italian origin. Thromb Haemost 1998 Apr;79(4):709-717.
  22. Surdhar GK, Enayat MS, Lawson S, Williams MD, Hill FG. Homozygous gene conversion in von Willebrand factor gene as a cause of type 3 von Willebrand disease and predisposition to inhibitor development. Blood 2001 Jul;98(1):248-250.
  23. Ginsburg D, Sadler JE. von Willebrand disease: a database of point mutations, insertions, and deletions. For the Consortium on von Willebrand Factor Mutations and Polymorphisms, and the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1993 Feb;69(2):177-184.
  24. Sadler JE, Ginsburg D. A database of polymorphisms in the von Willebrand factor gene and pseudogene. For the Consortium on von Willebrand Factor Mutations and Polymorphisms and the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 1993 Feb;69(2):185-191.
  25. 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 Apr;71(4):520-525.
  26. Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, Ingerslev J, Lee CA, Lillicrap D, Mannucci PM, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 2006 Oct;4(10):2103-2114.
  27. Castaman G, Federici AB, Rodeghiero F, Mannucci PM. Von Willebrand's disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica 2003 Jan;88(1):94-108.
  28. Meyer D, Fressinaud E, Gaucher C, Lavergne JM, Hilbert L, Ribba AS, 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 Jul;78(1):451-456.
  29. Budde U, Drewke E, Mainusch K, Schneppenheim R. Laboratory diagnosis of congenital von Willebrand disease. Semin Thromb Hemost 2002 Apr;28(2):173-189.
  30. Mannucci PM, Bloom AL, Larrieu MJ, Nilsson IM, West RR. Atherosclerosis and von Willebrand factor I. Prevalence of severe von Willebrand's disease in western Europe and Israel. Br J Haematol 1984 May;57(1):163-169.
  31. Weiss HJ, Ball AP, Mannucci PM. Incidence of severe von Willebrand's disease. N Engl J Med 1982 Jul;307(2):127.
  32. Berliner SA, Seligsohn U, Zivelin A, Zwang E, Sofferman G. A relatively high frequency of severe (type III) von Willebrand's disease in Israel. Br J Haematol 1986 Mar;62(3):535-543.
  33. Goodeve A, Eikenboom J, Castaman G, Rodeghiero F, Federici AB, Battle J, Meyer D, Mazurier C, Goudemand J, Schneppenheim R, et al. Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD). Blood 2007 Jan; 109(1):112-121.
  34. James PD, Notley C, Hegadorn C, Leggo J, Tuttle A, Tinlin S, Brown C, Andrews C, Labelle A, Chirinian Y, et al. The mutational spectrum of type 1 von Willebrand disease: results from a Canadian cohort study. Blood 2007 Jan; 109(1):145-154.
  35. Bodó I, Katsumi A, Tuley EA, Eikenboom JC, Dong Z, Sadler JE. Type 1 von Willebrand disease mutation Cys1149Arg causes intracellular retention and degradation of heterodimers: a possible general mechanism for dominant mutations of oligomeric proteins. Blood 2001 Nov;98(10):2973-2979.
  36. Casaña P, Martinez F, Haya S, Espinos C, Aznar JA. Association of the 3467C>T mutation (T1156M) in the von Willebrand's factor gene with dominant type 1 von Willebrand's disease. Ann Hematol 2001 Jul;80(7):381-383.
  37. Eikenboom JC, Matsushita T, Reitsma PH, Tuley EA, Castaman G, Briet E, Sadler JE. Dominant type 1 von Willebrand disease caused by mutated cysteine residues in the D3 domain of von Willebrand factor. Blood 1996 Oct;88(7):2433-2441.
  38. Gavazova S, Gill JC, Scott JP, Hillery CA, Friedman KD, Wetzel N, Jozwiak M, Haberichter SL, Christopherson P, Montgomery RR. A mutation in the D4 domain of von Willebrand factor (VWF) results in a variant of type 1 von Willebrand disease with accelerated in vivo VWF clearance. Blood 2002;100 (Suppl 1) 476:128a.
  39. Lethagen S, Isaksson C, Schaedel C, Holmberg L. Von Willebrand's disease caused by compound heterozygosity for a substitution mutation (T1156M) in the D3 domain of the von Willebrand factor and a stop mutation (Q2470X). Thromb Haemost 2002 Sep;88(3):421-426.
  40. Brown SA, Eldridge A, Collins PW, Bowen DJ. Increased clearance of von Willebrand factor antigen post-DDAVP in type 1 von Willebrand disease: is it a potential pathogenic process? J Thromb Haemost 2003 Aug;1(8):1714-1717.
  41. Casonato A, Pontara E, Sartorello F, Cattini MG, Sartori MT, Padrini R, Girolami A. Reduced von Willebrand factor survival in type Vicenza von Willebrand disease. Blood 2002 Jan;99(1):180-184.
  42. Schneppenheim R, Federici AB, Budde U, Castaman G, Drewke E, Krey S, Mannucci PM, Riesen G, Rodeghiero F, Zieger B, et al. 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 Jan;82(1):136-140.
  43. Gill JC, Endres-Brooks J, Bauer PJ, Marks WJ Jr, Montgomery RR. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987 Jun;69(6):1691-1695.
  44. Mannucci PM, Lattuada A, Castaman G, Lombardi R, Colibretti ML, Ciavarella N, Rodeghiero F. Heterogeneous phenotypes of platelet and plasma von Willebrand factor in obligatory heterozygotes for severe von Willebrand disease. Blood 1989 Nov;74(7):2433-2436.
  45. Sadler JE. Von Willebrand disease type 1: a diagnosis in search of a disease. Blood 2003 Mar;101(6):2089-2093.
  46. Ruggeri ZM, Pareti FI, Mannucci PM, Ciavarella N, Zimmerman TS. Heightened interaction between platelets and factor VIII/von Willebrand factor in a new subtype of von Willebrand's disease. N Engl J Med 1980 May;302(19):1047-1051.
  47. Dent JA, Galbusera M, Ruggeri ZM. Heterogeneity of plasma von Willebrand factor multimers resulting from proteolysis of the constituent subunit. J Clin Invest 1991 Sep;88(3):774-782.
  48. Ginsburg D, Konkle BA, Gill JC, Montgomery RR, Bockenstedt PL, Johnson TA, Yang AY. Molecular basis of human von Willebrand disease: analysis of platelet von Willebrand factor mRNA. Proc Natl Acad Sci U S A 1989 May;86(10):3723-3727.
  49. Lyons SE, Bruck ME, Bowie EJ, Ginsburg D. Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations. J Biol Chem 1992 Mar;267(7):4424-4430.
  50. Gaucher C, Diéval J, Mazurier C. Characterization of von Willebrand factor gene defects in two unrelated patients with type IIC von Willebrand disease. Blood 1994 Aug;84(4):1024-1030.
  51. Ruggeri ZM, Nilsson IM, Lombardi R, Holmberg L, Zimmerman TS. Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand's disease (type IIC).J Clin Invest 1982 Nov;70(5):1124-1127.
  52. Schneppenheim R, Thomas KB, Krey S, Budde U, Jessat U, Sutor AH, Zieger B. Identification of a candidate missense mutation in a family with von Willebrand disease type IIC. Hum Genet 1995 Jun;95(6):681-686.
  53. Kinoshita S, Harrison J, Lazerson J, Abildgaard CF. A new variant of dominant type II von Willebrand's disease with aberrant multimeric pattern of factor VIII-related antigen (type IID). Blood 1984 Jun;63(6):1369-1371.
  54. Schneppenheim R, Brassard J, Krey S, Budde U, Kunicki TJ, Holmberg L, Ware J, Ruggeri ZM. Defective dimerization of von Willebrand factor subunits due to a Cys→ Arg mutation in type IID von Willebrand disease. Proc Natl Acad Sci U S A 1996 Apr;93(8):3581-3586.
  55. Schneppenheim R, Budde U, Ruggeri ZM. A molecular approach to the classification of von Willebrand disease. Best Pract Res Clin Haematol 2001 Jun;14(2):281-298.
  56. Zimmerman TS, Dent JA, Ruggeri ZM, Nannini LH. Subunit composition of plasma von Willebrand factor. Cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (types IIC, IID, and IIE). J Clin Invest 1986 Mar;77(3):947-951.
  57. Sutherland JJ, O'Brien LA, Lillicrap D, Weaver DF. Molecular modeling of the von Willebrand factor A2 domain and the effects of associated type 2A von Willebrand disease mutations. J Mol Model (Online) 2004 Aug;10(4):259-270.
  58. Ruggeri ZM, Zimmerman TS. Variant von Willebrand's disease: characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets. J Clin Invest 1980 Jun;65(6):1318-1325.
  59. Lankhof H, Damas C, Schiphorst ME, IJsseldijk MJ, Bracke M, Sixma JJ, Vink T, de Groot PG. Functional studies on platelet adhesion with recombinant von Willebrand factor type 2B mutants R543Q and R543W under conditions of flow. Blood 1997 Apr;89(8):2766-2772.
  60. Giles AR, Hoogendoorn H, Benford K. Type IIB von Willebrand's disease presenting as thrombocytopenia during pregnancy. Br J Haematol 1987 Nov;67(3):349-353.
  61. Hultin MB, Sussman II. Postoperative thrombocytopenia in type IIB von Willebrand disease. Am J Hematol 1990 Jan;33(1):64-68.
  62. Rick ME, Williams SB, Sacher RA, McKeown LP. Thrombocytopenia associated with pregnancy in a patient with type IIB von Willebrand's disease. Blood 1987 Mar;69(3):786-789.
  63. Casonato A, Fabris F, Girolami A. Platelet aggregation and pseudothrombocytopenia induced by 1-desamino-8-D-arginine vasopressin (DDAVP) in type IIB von Willebrand's disease patient. Eur J Haematol 1990 Jul;45(1):36-42.
  64. Casonato A, Sartori MT, de Marco L, Girolami A. 1-Desamino-8-D-arginine vasopressin (DDAVP) infusion in type IIB von Willebrand's disease: shortening of bleeding time and induction of a variable pseudothrombocytopenia. Thromb Haemost 1990 Aug;64(1):117-120.
  65. Cooney KA, Nichols WC, Bruck ME, Bahou WF, Shapiro AD, Bowie EJ, Gralnick HR, Ginsburg D. The molecular defect in type IIB von Willebrand disease. Identification of four potential missense mutations within the putative GpIb binding domain. J Clin Invest 1991 Apr;87(4):1227-1233.
  66. Kroner PA, Kluessendorf ML, Scott JP, Montgomery RR. Expressed full-length von Willebrand factor containing missense mutations linked to type IIB von Willebrand disease shows enhanced binding to platelets. Blood 1992 Apr;79(8):2048-2055.
  67. Meyer D, Fressinaud E, Hilbert L, Ribba AS, Lavergne JM, Mazurier C. Type 2 von Willebrand disease causing defective von Willebrand factor-dependent platelet function. Best Pract Res Clin Haematol 2001 Jun;14(2):349-364.
  68. Randi AM, Rabinowitz I, Mancuso DJ, Mannucci PM, Sadler JE. Molecular basis of von Willebrand disease type IIB. Candidate mutations cluster in one disulfide loop between proposed platelet glycoprotein Ib binding sequences. J Clin Invest 1991 Apr;87(4): 1220-1226.
  69. Huizinga EG, Tsuji S, Romijn RA, Schiphorst ME, de Groot PG, Sixma JJ, Gros P. Structures of glycoprotein Ib alpha and its complex with von Willebrand factor A1 domain. Science 2002 Aug;297(5584):1176-1179.
  70. Hillery CA, Mancuso DJ, Sadler JE, Ponder JW, Jozwiak MA, Christopherson PA, Cox Gill J, Scott JP, Montgomery RR. 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 Mar;91(5):1572-1581.
  71. Mancuso DJ, Kroner PA, Christopherson PA, Vokac EA, Cox Gill J, Montgomery RR. 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 Oct;88(7):2559-2568.
  72. Rabinowitz I, Tuley EA, Mancuso DJ, Randi AM, Firkin BG, Howard MA, Sadler JE. von Willebrand disease type B: a missense mutation selectively abolishes ristocetin-induced von Willebrand factor binding to platelet glycoprotein Ib. Proc Natl Acad Sci U S A 1992 Oct;89(20):9846-9849.
  73. Ribba AS, Loisel I, Lavergne JM, Juhan-Vague I, Obert B, Cherel G, Meyer D, Girma JP. Ser968Thr mutation within the A3 domain of von Willebrand factor (VWF) in two related patients leads to a defective binding of VWF to collagen. Thromb Haemost 2001 Sep;86(3):848-854.
  74. 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 Jan;75(1):20-26.
  75. Montgomery RR, Hathaway WE, Johnson J, Jacobson L, Muntean W. A variant of von Willebrand's disease with abnormal expression of factor VIII procoagulant activity. Blood 1982 Jul;60(1):201-207.
  76. Nishino M, Girma JP, Rothschild C, Fressinaud E, Meyer D. New variant of von Willebrand disease with defective binding to factor VIII. Blood 1989 Oct;74(5):1591-1599.
  77. Mazurier C, Meyer D. Factor VIII binding assay of von Willebrand factor and the diagnosis of type 2N von Willebrand disease—results of an international survey. On behalf of the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the ISTH. Thromb Haemost 1996 Aug;76(2):270-274.
  78. Schneppenheim R, Budde U, Krey S, Drewke E, Bergmann F, Lechler E, Oldenburg J, Schwaab R. Results of a screening for von Willebrand disease type 2N in patients with suspected haemophilia A or von Willebrand disease type 1. Thromb Haemost 1996 Oct;76(4):598-602.
  79. Kroner PA, Friedman KD, Fahs SA, Scott JP, Montgomery RR. Abnormal binding of factor VIII is linked with the substitution of glutamine for arginine 91 in von Willebrand factor in a variant form of von Willebrand disease. J Biol Chem 1991 Oct;266(29):19146-19149.
  80. 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 Haematol 2001 Jun;14(2):337-347.
  81. Allen S, Abuzenadah AM, Blagg JL, Hinks J, Nesbitt IM, Goodeve AC, Gursel T, Ingerslev J, Peake IR, Daly ME. Two novel type 2N von Willebrand disease-causing mutations that result in defective factor VIII binding, multimerization, and secretion of von Willebrand factor. Blood 2000 Mar;95(6):2000-2007.
  82. Hilbert L, d'Oiron R, Fressinaud E, Meyer D, Mazurier C. INSERM Network on Molecular Abnormalities in von Willebrand Disease. First identification and expression of a type 2N von Willebrand disease mutation (E1078K) located in exon 25 of von Willebrand factor gene. J Thromb Haemost 2004 Dec;2(12):2271-2273.
  83. Hilbert L, Jorieux S, Proulle V, Favier R, Goudemand J, Parquet A, Meyer D, Fressinaud E, Mazurier C. INSERM Network on Molecular Abnormalities in von Willebrand Disease. Two novel mutations, Q1053H and C1060R, located in the D3 domain of von Willebrand factor, are responsible for decreased FVIII-binding capacity. Br J Haematol 2003 Feb;120(4):627-632.
  84. Italian Working Group. Spectrum of von Willebrand's disease: a study of 100 cases. Br J Haematol 1977 Jan;35(1):101-112.
  85. Lak M, Peyvandi F, Mannucci PM. Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease. Br J Haematol 2000 Dec;111(4):1236-1239.
  86. Zhang ZP, Lindstedt M, Falk G, Blombäck M, Egberg N, Anvret M. Nonsense mutations of the von Willebrand factor gene in patients with von Willebrand disease type III and type I. Am J Hum Genet 1992 Oct;51(4):850-858.
  87. Baronciani L, Cozzi G, Canciani MT, Peyvandi F, Srivastava A, Federici AB, Mannucci PM. Molecular defects in type 3 von Willebrand disease: updated results from 40 multiethnic patients. Blood Cells Mol Dis 2003 May;30(3):264-270.
  88. Eikenboom JC. Congenital von Willebrand disease type 3: clinical manifestations, pathophysiology and molecular biology. Best Pract Res Clin Haematol 2001 Jun;14(2):365-379.
  89. Mannucci PM, Federici AB. Antibodies to von Willebrand factor in von Willebrand disease. Adv Exp Med Biol 1995;386:87-92.
  90. Mannucci PM, Ruggeri ZM, Ciavarella N, Kazatchkine MD, Mowbray JF. Precipitating antibodies to factor VIII/von Willebrand factor in von Willebrand's disease: effects on replacement therapy. Blood 1981 Jan;57(1):25-31.
  91. Nitu-Whalley IC, Riddell A, Lee CA, Pasi KJ, Owens D, Enayat MS, Perkins SJ, Jenkins PV. Identification of type 2 von Willebrand disease in previously diagnosed type 1 patients: a reappraisal using phenotypes, genotypes and molecular modelling. Thromb Haemost 2000 Dec;84(6):998-1004.
  92. Mannucci PM, Lombardi R, Castaman G, Dent JA, Lattuada A, Rodeghiero F, Zimmerman TS. Von Willebrand disease "Vicenza" with larger-than-normal (supranormal) von Willebrand factor multimers. Blood 1988 Jan;71(1):65-70.
  93. Sadler JE. New concepts in von Willebrand disease. Annu Rev Med 2005;56:173-191.
  94. Castaman G, Rodeghiero F, Mannucci PM. The elusive pathogenesis of von Willebrand disease Vicenza. Blood 2002 Jun;99(11):4243-4244.
  95. Bodó I, Katsumi A, Tuley EA, Schlammadinger A, Boda Z, Sadler JE. Mutations causing dominant type 1 von Willebrand disease with high penetrance. Blood 1999;94 (Suppl 1)1661:373a.
  96. Castaman G, Eikenboom JC, Missiaglia E, Rodeghiero F. Autosomal dominant type 1 von Willebrand disease due to G3639T mutation (C1130F) in exon 26 of von Willebrand factor gene: description of five Italian families and evidence for a founder effect. Br J Haematol 2000 Mar;108(4):876-879.
  97. Eikenboom J, Van Marion V, Putter H, Goodeve A, Rodeghiero F, Castaman G, Federici AB, Batlle J, Meyer D, Mazurier C, et al. Linkage analysis in families diagnosed with type 1 von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 VWD. J Thromb Haemost 2006 Apr;4(4):774-782.
  98. James PD, Paterson AD, Notley C, Cameron C, Hegadorn C, Tinlin S, Brown C, O'Brien L, Leggo J, Lillicrap D. Genetic linkage and association analysis in type 1 von Willebrand disease: results from the Canadian type 1 VWD study. J Thromb Haemost 2006 Apr;4(4):783-792.
  99. Mannucci PM, Lombardi R, Bader R, Vianello L, Federici AB, Solinas S, Mazzucconi MG, Mariani G. Heterogeneity of type I von Willebrand disease: evidence for a subgroup with an abnormal von Willebrand factor. Blood 1985 Oct;66(4):796-802.
  100. Casaña P, Martinez F, Haya S, Espinos C, Aznar JA. Significant linkage and non-linkage of type 1 von Willebrand disease to the von Willebrand factor gene. Br J Haematol 2001 Dec;115(3):692-700.
  101. Castaman G, Eikenboom JC, Bertina RM, Rodeghiero F. Inconsistency of association between type 1 von Willebrand disease phenotype and genotype in families identified in an epidemiological investigation. Thromb Haemost 1999 Sep;82(3):1065-1070.
  102. Miller CH, Graham JB, Goldin LR, Elston RC. Genetics of classic von Willebrand's disease. I. Phenotypic variation within families. Blood 1979 Jul;54(1):117-136.
  103. Souto JC, Almasy L, Borrell M, Garí M, Martínez E, Mateo J, Stone WH, Blangero J, Fontcuberta J. Genetic determinants of hemostasis phenotypes in Spanish families. Circulation 2000 Apr;101 (13):1546-1551.
  104. Vossen CY, Hasstedt SJ, Rosendaal FR, Callas PW, Bauer KA, Broze GJ, Hoogendoorn H, Long GL, Scott BT, Bovill EG. Heritability of plasma concentrations of clotting factors and measures of a prethrombotic state in a protein C-deficient family. J Thromb Haemost 2004 Feb;2(2):242-247.
  105. de Lange M, Snieder H, Ariens RA, Spector TD, Grant PJ. The genetics of haemostasis: a twin study. Lancet 2001 Jan;357(9250):101-105.
  106. Ørstavik KH, Magnus P, Reisner H, Berg K, Graham JB, Nance W. Factor VIII and factor IX in a twin population. Evidence for a major effect of ABO locus on factor VIII level. Am J Hum Genet 1985 Jan;37(1):89-101.
  107. Harrap SB, Hopper JL. Genetics of haemostasis. Lancet 2001 Jan;357(9250):83-84.
  108. de Visser MC, Sandkuijl LA, Lensen RP, Vos HL, Rosendaal FR, Bertina RM. Linkage analysis of factor VIII and von Willebrand factor loci as quantitative trait loci. J Thromb Haemost 2003 Aug;1(8):1771-1776.
  109. O'Donnell J, Boulton FE, Manning RA, Laffan MA. Genotype at the secretor blood group locus is a determinant of plasma von Willebrand factor level. Br J Haematol 2002 Feb;116(2):350-356.
  110. Souto JC, Almasy L, Soria JM, Buil A, Stone W, Lathrop M, Blangero J, Fontcuberta J. Genomewide linkage analysis of von Willebrand factor plasma levels: results from the GAIT project. Thromb Haemost 2003 Mar;89(3):468-474.
  111. Castaman G, Novella E, Castiglia E, Eikenboom JC, Rodeghiero F. A novel family with recessive von Willebrand disease due to compound heterozygosity for a splice site mutation and a missense mutation in the von Willebrand factor gene. Thromb Res 2002 Jan;105(2):135-138.
  112. Zhang Z, Lindstedt M, Blomback M, Anvret M. Effects of the mutant von Willebrand factor gene in von Willebrand disease. Hum Genet 1995 Oct;96(4):388-394.
  113. Schneppenheim R, Krey S, Bergmann F, Bock D, Budde U, Lange M, Linde R, Mittler U, Meili E, Mertes G, et al. Genetic heterogeneity of severe von Willebrand disease type III in the German population. Hum Genet 1994 Dec;94(6):640-652.
  114. Eikenboom JC, Reitsma PH, Peerlinck KM, Briet E. Recessive inheritance of von Willebrand's disease type I. Lancet 1993 Apr;341(8851):982-986.
  115. Inbal A, Kornbrot N, Zivelin A, Shaklai M, Seligsohn U. The inheritance of type I and type III von Willebrand's disease in Israel: linkage analysis, carrier detection and prenatal diagnosis using three intragenic restriction fragment length polymorphisms. Blood Coagul Fibrinolysis 1992 Apr;3(2):167-177.
  116. Nichols WC, Lyons SE, Harrison JS, Cody RL, Ginsburg D. Severe von Willebrand disease due to a defect at the level of von Willebrand factor mRNA expression: detection by exonic PCR-restriction fragment length polymorphism analysis. Proc Natl Acad Sci USA 1991 May;88(9):3857-3861.
  117. Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H, Meyer D, Rodeghiero F, Sadler JE, Subcommittee on von Willebrand Factor. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000 Aug;84(2):345-349.
  118. Veyradier A, Jenkins CS, Fressinaud E, Meyer D. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost 2000 Aug;84(2):175-182.
  119. 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 Mar;67(3):758-761.
  120. Rauch R, Budde U, Koch A, Girisch M, Hofbeck M. Acquired von Willebrand syndrome in children with patent ductus arteriosus. Heart 2002 Jul;88(1):87-88.
  121. Veyradier A, Nishikubo T, Humbert M, Wolf M, Sitbon O, Simonneau G, Girma JP, Meyer D. Improvement of von Willebrand factor proteolysis after prostacyclin infusion in severe pulmonary arterial hypertension. Circulation 2000 Nov;102(20):2460-2462.
  122. Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F, Bauters A, Decoene C, Goudemand J, Prat A, et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003 Jul;349(4):343-349.
  123. Budde U, Scharf RE, Franke P, Hartmann-Budde K, Dent J, Ruggeri ZM. Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood 1993 Sep;82(6):1749-1757.
  124. Mazzucconi MG, Ferrari A, Solinas S, Vitale A, Chistolini A, Federici AB, Mandelli F. Studies of von Willebrand factor in essential thrombocythemia patients treated with alpha-2b recombinant interferon. Haemostasis 1991; 21(3):135-140.
  125. van Genderen PJ, Budde U, Michiels JJ, van Strik R, van Vliet HH. The reduction of large von Willebrand factor multimers in plasma in essential thrombocythaemia is related to the platelet count. Br J Haematol 1996 Jun;93(4):962-965.
  126. Tefferi A, Nichols WL. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis, and treatment. Am J Med 1997 Dec;103(6):536-540.
  127. Dalton RG, Dewar MS, Savidge GF, Kernoff PB, Matthews KB, Greaves M, Preston FE. Hypothyroidism as a cause of acquired von Willebrand's disease. Lancet 1987 May;1(8540):1007-1009.
  128. Lo B, Fijnheer R, Castigliego D, Borst C, Kalkman CJ, Nierich AP. Activation of hemostasis after coronary artery bypass grafting with or without cardiopulmonary bypass. Anesth Analg 2004 Sep;99(3):634-40, table.
  129. Cortellaro M, Boschetti C, Cofrancesco E, Zanussi C, Catalano M, de Gaetano G, Gabrielli L, Lombardi B, Specchia G, Tavazzi L, et al. The PLAT Study: hemostatic function in relation to atherothrombotic ischemic events in vascular disease patients. Principal results. Progetto Lombardo Atero-Trombosi (PLAT) Study Group. Arterioscler Thromb 1992 Sep;12(9):1063-1070.
  130. Folsom AR, Wu KK, Rosamond WD, Sharrett AR, Chambless LE. Prospective study of hemostatic factors and incidence of coronary heart disease: the Atherosclerosis Risk in Communities (ARIC) Study. Circulation 1997 Aug;96(4):1102-1108.
  131. Thompson SG, Kienast J, Pyke SD, Haverkate F, van de Loo JC. 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 Mar;332(10):635-641.
  132. Conway DS, Pearce LA, Chin BS, Hart RG, Lip GY. 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 Jul;107(25):3141-3145.
  133. Moake JL. von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura. Semin Hematol 2004 Jan;41(1):4-14.
  134. Tsai HM. Shear stress and von Willebrand factor in health and disease. Semin Thromb Hemost 2003 Oct;29(5):479-488.
  135. Koster T, Blann AD, Briët E, Vandenbroucke JP, Rosendaal FR. Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet 1995 Jan;345(8943):152-155.
  136. Federici AB. Clinical diagnosis of von Willebrand disease. Haemophilia 2004 Oct;10 (Suppl 4):169-176.
  137. Silwer J. Von Willebrand's disease in Sweden. Acta Paediatr Scand 1973;(Suppl 238):1-159.
  138. Sramek A, Eikenboom JC, Briet E, Vandenbroucke JP, Rosendaal FR. Usefulness of patient interview in bleeding disorders. Arch Intern Med 1995 Jul;155(13):1409-1415.
  139. Drews CD, Dilley AB, Lally C, Beckman MG, Evatt B. Screening questions to identify women with von Willebrand disease. J Am Med Womens Assoc 2002;Fall;57(4):217-218.
  140. Mauser Bunschoten EP, van Houwelingen JC, Sjamsoedin Visser EJ, van Dijken PJ, Kok AJ, Sixma JJ. Bleeding symptoms in carriers of hemophilia A and B. Thromb Haemost 1988 Jun;59(3):349-352.
  141. Woods AI, Meschengieser SS, Blanco AN, Salviu MJ, Farias CE, Kempfer AC, Lazzari MA. Clinical features and laboratory patterns in a cohort of consecutive Argentinian patients with von Willebrand's disease. Haematologica 2001 Apr;86(4):420-427.
  142. Ziv O, Ragni MV. Bleeding manifestations in males with von Willebrand disease. Haemophilia 2004 March;10(2):162-168.
  143. Nosek-Cenkowska B, Cheang MS, Pizzi NJ, Israels ED, Gerrard JM. Bleeding/bruising symptomatology in children with and without bleeding disorders. Thromb Haemost 1991 Mar;65(3):237-241.
  144. Wahlberg T, Blomback M, Hall P, Axelsson G. Application of indicators, predictors and diagnostic indices in coagulation disorders. II. Evaluation of laboratory variables with continuous test results. Methods Inf Med 1980 Oct;19(4):201-205.
  145. Kirtava A, Drews C, Lally C, Dilley A, Evatt B. Medical, reproductive and psychosocial experiences of women diagnosed with von Willebrand's disease receiving care in haemophilia treatment centres: a case-control study. Haemophilia 2003 May;9(3):292-297.
  146. Dilley A, Drews C, Miller C, Lally C, Austin H, Ramaswamy D, Lurye D, Evatt B. von Willebrand disease and other inherited bleeding disorders in women with diagnosed menorrhagia. Obstet Gynecol 2001 Apr;97(4):630-636.
  147. Edlund M, Blomback M, von Schoultz B, Andersson O. On the value of menorrhagia as a predictor for coagulation disorders. Am J Hematol 1996 Dec;53(4):234-238.
  148. Goodman-Gruen D, Hollenbach K. The prevalence of von Willebrand disease in women with abnormal uterine bleeding. J Womens Health Gend Based Med 2001 Sep;10(7):677-680.
  149. James AH, Lukes AS, Brancazio LR, Thames E, Ortel TL. Use of a new platelet function analyzer to detect von Willebrand disease in women with menorrhagia. Am J Obstet Gynecol 2004 Aug;191(2):449-455.
  150. Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Frequency of inherited bleeding disorders in women with menorrhagia. Lancet 1998 Feb;(9101):485-489.
  151. Philipp CS, Dilley A, Miller CH, Evatt B, Baranwal A, Schwartz R, Bachmann G, Saidi P. Platelet functional defects in women with unexplained menorrhagia. J Thromb Haemost 2003 Mar;1(3):477-484.
  152. Woo YL, White B, Corbally R, Byrne M, O'Connell N, O'Shea E, Sheppard BL, Bonnar J, Smith OP. von Willebrand's disease: an important cause of dysfunctional uterine bleeding. Blood Coagul Fibrinolysis 2002 Mar;13(2):89-93.
  153. Warner PE, Critchley HO, Lumsden MA, Campbell-Brown M, Douglas A, Murray GD. Menorrhagia I: measured blood loss, clinical features, and outcome in women with heavy periods: a survey with follow-up data. Am J Obstet Gynecol 2004 May;190(5):1216-1223.
  154. Tosetto A, Rodeghiero F, Castaman G, Goodeve A, Federici AB, Batlle J, Meyer D, Fressinaud E, Mazurier C, Goudemand J, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006 Apr;4(4):766-773.
  155. Rodeghiero F, Castaman G, Tosetto A, Batlle J, Baudo F, Cappelletti A, Casana P, De Bosch N, Eikenboom JC, Federici AB, et al. The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study. J Thromb Haemost 2005 Dec;3(12):2619-2626.
  156. Castaman G, Rodeghiero F, Tosetto A, Cappelletti A, Baudo F, Eikenboom JC, Federici AB, Lethagen S, Linari S, Lusher J, et al. Hemorrhagic symptoms and bleeding risk in obligatory carriers of type 3 von Willebrand disease: an international, multicenter study. J Thromb Haemost 2006 Oct;4(10):2164-2169.
  157. Kumar S, Pruthi RK, Nichols WL. Acquired von Willebrand's syndrome: a single institution experience. Am J Hematol 2003 Apr;72(4):243-247.
  158. Rodgers RP, Levin J. A critical reappraisal of the bleeding time. Semin Thromb Hemost 1990 Jan;16(1):1-20.
  159. Cattaneo M, Federici AB, Lecchi A, Agati B, Lombardi R, Stabile F, Bucciarelli P. Evaluation of the PFA-100 system in the diagnosis and therapeutic monitoring of patients with von Willebrand disease. Thromb Haemost 1999 Jul;82(1):35-39.
  160. Cattaneo M, Lecchi A, Agati B, Lombardi R, Zighetti ML. Evaluation of platelet function with the PFA-100 system in patients with congenital defects of platelet secretion. Thromb Res 1999 Nov;96(3):213-217.
  161. Fressinaud E, Veyradier A, Truchaud F, Martin I, Boyer-Neumann C, Trossaert M, Meyer D. Screening for von Willebrand disease with a new analyzer using high shear stress: a study of 60 cases. Blood 1998 Feb;91(4):1325-1331.
  162. Quiroga T, Goycoolea M, Munoz B, Morales M, Aranda E, Panes O, Pereira J, Mezzano D. Template bleeding time and PFA-100 have low sensitivity to screen patients with hereditary mucocutaneous hemorrhages: comparative study in 148 patients. J Thromb Haemost 2004 Jun;2(6):892-898.
  163. Dean JA, Blanchette VS, Carcao MD, Stain AM, Sparling CR, Siekmann J, Turecek PL, Lillicrap D, Rand ML. von Willebrand disease in a pediatric-based population—comparison of type 1 diagnostic criteria and use of the PFA-100® and a von Willebrand factor/collagen-binding assay. Thromb Haemost 2000 Sep;84(3):401-409.
  164. 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 Sep;90(3):483-490.
  165. Schlammadinger A, Kerenyi A, Muszbek L, Boda Z. Comparison of the O'Brien filter test and the PFA-100 platelet analyzer in the laboratory diagnosis of von Willebrand's disease. Thromb Haemost 2000 Jul;84(1):88-92.
  166. Cariappa R, Wilhite TR, Parvin CA, Luchtman-Jones L. Comparison of PFA-100 and bleeding time testing in pediatric patients with suspected hemorrhagic problems. J Pediatr Hematol Oncol 2003 Jun;25(6):474-479.
  167. Kunicki TJ, Baronciani L, Canciani MT, Gianniello F, Head SR, Mondala TS, Salomon DR, Federici AB. An association of candidate gene haplotypes and bleeding severity in von Willebrand disease type 2A, 2B, and 2M pedigrees. J Thromb Haemost 2006 Jan;4(1):137-147.
  168. Michiels JJ, van de Velde A, van Vliet HH, van der Planken M, Schroyens W, Berneman Z. Response of von Willebrand factor parameters to desmopressin in patients with type 1 and type 2 congenital von Willebrand disease: diagnostic and therapeutic implications. Semin Thromb Hemost 2002 Apr;28(2):111-132.
  169. Federici AB, Canciani MT, Forza I, Mannucci PM, Marchese P, Ware J, Ruggeri ZM. A sensitive ristocetin co-factor activity assay with recombinant glycoprotein Ib alpha for the diagnosis of patients with low von Willebrand factor levels. Haematologica 2004 Jan;89(1):77-85.
  170. Vanhoorelbeke K, Cauwenberghs N, Vauterin S, Schlammadinger A, Mazurier C, Deckmyn H. A reliable and reproducible ELISA method to measure ristocetin cofactor activity of von Willebrand factor. Thromb Haemost 2000 Jan;83(1):107-113.
  171. Vanhoorelbeke K, Pareyn I, Schlammadinger A, Vauterin S, Hoylaerts MF, Arnout J, Deckmyn H. Plasma glycocalicin as a source of GPIb-alpha in the von Willebrand factor ristocetin cofactor ELISA. Thromb Haemost 2005 Jan;93(1):165-171.
  172. Murdock PJ, Woodhams BJ, Matthews KB, Pasi KJ, Goodall AH. von Willebrand factor activity detected in a monoclonal antibody-based ELISA: an alternative to the ristocetin cofactor platelet agglutination assay for diagnostic use. Thromb Haemost 1997 Oct; 78(4):1272-1277.
  173. Caron C, Hilbert L, Vanhoorelbeke K, Deckmyn H, Goudemand J, Mazurier C. Measurement of von Willebrand factor binding to a recombinant fragment of glycoprotein Ibα in an enzyme-linked immunosorbent assay-based method: performances in patients with type 2B von Willebrand disease. Br J Haematol 2006; 133(6):655.
  174. Fischer BE, Thomas KB, Dorner F. von Willebrand factor: measuring its antigen or function? Correlation between the level of antigen, activity, and multimer size using various detection systems. Thromb Res 1998 Jul;91(1):39-43.
  175. Favaloro EJ, Bonar R, Kershaw G, Sioufi J, Hertzberg M, Street A, Lloyd J, Marsden K. Laboratory diagnosis of von Willebrand's disorder: quality and diagnostic improvements driven by peer review in a multilaboratory test process. Haemophilia 2004 May;10(3):232-242.
  176. Favaloro EJ, Thom J, Baker R, Australasian Society for Thrombosis and Haemostasis (ASTH) Emerging Technology Group. Assessment of current diagnostic practice and efficacy in testing for von Willebrand's disorder: results from the second Australasian multilaboratory survey. Blood Coagul Fibrinolysis 2000 Dec;11(8):729-737.
  177. Hayes TE, Brandt JT, Chandler WL, Eby CS, Kottke-Marchant K, Krishnan J, Lefkowitz JB, Olson JD, Rund CR, Van Cott EM, et al. External peer review quality assurance testing in von Willebrand disease: the recent experience of the United States College of American Pathologists proficiency testing program. Semin Thromb Hemost 2006 Jul;32(5):499-504.
  178. Kitchen S, Jennings I, Woods TA, Kitchen DP, Walker ID, Preston FE. Laboratory tests for measurement of von Willebrand factor show poor agreement among different centers: results from the United Kingdom National External Quality Assessment Scheme for Blood Coagulation. Semin Thromb Hemost 2006 Jul;32(5):492-498.
  179. Meijer P, Haverkate F. An external quality assessment program for von Willebrand factor laboratory analysis: an overview from the European concerted action on thrombosis and disabilities foundation. Semin Thromb Hemost 2006 Jul;32(5):485-491.
  180. Budde U, Pieconka A, Will K, Schneppenheim R. Laboratory testing for von Willebrand disease: contribution of multimer analysis to diagnosis and classification. Semin Thromb Hemost 2006 Jul;32(5):514-521.
  181. Studt JD, Budde U, Schneppenheim R, Eisert R, von Depka Prondzinski M, Ganser A, Barthels M. Quantification and facilitated comparison of von Willebrand factor multimer patterns by densitometry. Am J Clin Pathol 2001 Oct;116(4):567-574.
  182. Scott JP, Montgomery RR. The rapid differentiation of type IIb von Willebrand's disease from platelet-type (pseudo-) von Willebrand's disease by the "neutral" monoclonal antibody binding assay. Am J Clin Pathol 1991 Dec;96(6):723-728.
  183. Favaloro EJ. Detection of von Willebrand disorder and identification of qualitative von Willebrand factor defects: Direct comparison of commercial ELISA-based von Willebrand factor activity options. Am J Clin Pathol 2000 Oct;114(4):608-618.
  184. Neugebauer BM, Goy C, Budek I, Seitz R. Comparison of two von Willebrand factor collagen-binding assays with different binding affinities for low, medium, and high multimers of von Willebrand factor. Semin Thromb Hemost 2002 Apr;28(2):139-148.
  185. Favaloro EJ. Collagen binding assay for von Willebrand factor (VWF:CBA): detection of von Willebrands disease (VWD), and discrimination of VWD subtypes, depends on collagen source. Thromb Haemost 2000 Jan;83(1):127-135.
  186. Favaloro EJ, Henniker A, Facey D, Hertzberg M. Discrimination of von Willebrands disease (VWD) subtypes: direct comparison of von Willebrand factor:collagen binding assay (VWF:CBA) with monoclonal antibody (MAB) based VWF-capture systems. Thromb Haemost 2000 Oct;84(4):541-547.
  187. Kroner PA, Foster PA, Fahs SA, Montgomery RR. The defective interaction between von Willebrand factor and factor VIII in a patient with type 1 von Willebrand disease is caused by substitution of Arg19 and His54 in mature von Willebrand factor. Blood 1996 Feb;87(3):1013-1021.
  188. Rodgers SE, Lerda NV, Favaloro EJ, Duncan EM, Casey GJ, Quinn DM, Hertzberg M, Lloyd JV. Identification of von Willebrand disease type 2N (Normandy) in Australia: a cross-laboratory investigation using different methods. Am J Clin Pathol 2002 Aug;118(2):269-276.
  189. Favaloro EJ, Lillicrap D, Lazzari MA, Cattaneo M, Mazurier C, Woods A, Meschengieser S, Blanco A, Kempfer AC, Hubbard A, et al. von Willebrand disease: laboratory aspects of diagnosis and treatment. Haemophilia 2004 Oct;10 Suppl 4:164-168.
  190. Federici AB, Canciani MT, 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 Dec;84(6):1127-1128.
  191. Veyradier A, Fressinaud E, Meyer D. Laboratory diagnosis of von Willebrand disease. Int J Clin Lab Res 1998;28(4):201-210.
  192. Tomasulo PA, Richards W, Bailey M, Gajewski M, Aster RJ, Lazerson J. Preselection of donors to improve the quality of cryoprecipitate. Am J Hematol 1980 Apr;8(2):191-196.
  193. National Committee for Clinical Laboratory Standards (NCCLS). Assays of von Willebrand factor antigen and ristocetin cofactor activity: approved guideline. Wayne, PA: NCCLS; 2002. H51-A.
  194. Sadler JE, Rodeghiero F, ISTH SSC Subcommittee on von Willebrand Factor. Provisional criteria for the diagnosis of VWD type 1. J Thromb Haemost 2005 Apr;3(4):775-777.
  195. Favaloro EJ, Soltani S, McDonald J, Grezchnik E, Easton L, Favaloro JW. Reassessment of ABO blood group, sex, and age on laboratory parameters used to diagnose von Willebrand disorder: potential influence on the diagnosis vs the potential association with risk of thrombosis. Am J Clin Pathol 2005 Dec;124(6):910-917.
  196. Nitu-Whalley IC, Lee CA, Griffioen A, Jenkins PV, Pasi KJ. Type 1 von Willebrand disease—a clinical retrospective study of the diagnosis, the influence of the ABO blood group and the role of the bleeding history. Br J Haematol 2000 Feb;108(2):259-264.
  197. Ginsburg D. Molecular genetics of von Willebrand disease. Thromb Haemost 1999 Aug;82(2):585-591.
  198. Montgomery RR, Kroner PA. Von Willebrand disease: a common pediatric disorder. Pediatr Ann 2001 Sep;30(9):534-540.
  199. Sadler JE. Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem 1998;67:395-424.
  200. Montgomery RR. Structure and function of von Willebrand factor. In: Colman RW, Hirsh J, Marder VJ, Clowes AW, George JN, eds. Hemostasis and thrombosis: basic principles and clinical practice. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2001. 249-274.
  201. Montgomery RR, Coller BS. von Willebrand disease. In: Colman RW, Hirsh J, Marder VJ, Salzman EW, eds. Hemostasis and thrombosis: basic principles and clinical practice. 3rd ed. Philadelphia: JB Lippincott Company; 1994. 134-168.
  202. van Genderen PJ, Boertjes RC, van Mourik JA. Quantitative analysis of von Willebrand factor and its propeptide in plasma in acquired von Willebrand syndrome. Thromb Haemost 1998 Sep;80(3):495-498.
  203. Shelton-Inloes BB, Chehab FF, Mannucci PM, Federici AB, Sadler JE. Gene deletions correlate with the development of alloantibodies in von Willebrand disease. J Clin Invest 1987 May;79(5):1459-1465.
  204. Sadler JE. Slippery criteria for von Willebrand disease type 1. J Thromb Haemost 2004 Oct;2(10):1720-1723.
  205. National Committee for Clinical Laboratory Standards (NCCLS). Collection, transport, and processing of blood specimens for testing plasma-based coagulation assays; approved guideline. Wayne, PA: NCCLS; 2003. H21-A4.
  206. Favaloro EJ, Nair SC, Forsyth CJ. Collection and transport of samples for laboratory testing in von Willebrand's disease (VWD): time for a reappraisal? Thromb Haemost 2001 Dec;86(6):1589-1590.
  207. Favaloro EJ, Soltani S, McDonald J. Potential laboratory misdiagnosis of hemophilia and von Willebrand disorder owing to cold activation of blood samples for testing. Am J Clin Pathol 2004 Nov;122(5):686-692.
  208. Preston FE, Kitchen S. Quality control and factor VIII assays. Haemophilia 1998 Jul;4(4):651-653.
  209. Preston FE, Kitchen S, Jennings I, Woods TA, Makris M. SSC/ISTH classification of hemophilia A: can hemophilia center laboratories achieve the new criteria? J Thromb Haemost 2004 Feb;2(2):271-274.
  210. van den Besselaar AM, Haas FJ, Kuypers AW. Harmonisation of factor VIII:C assay results: study within the framework of the Dutch project ‘Calibration 2000’. Br J Haematol 2006 Jan;132(1):75-79.
  211. Berntorp E, Petrini P. Long-term prophylaxis in von Willebrand disease. Blood Coagul Fibrinolysis 2005 Apr;16 (Suppl 1):S23-S26.
  212. Sumner M, Williams J. Type 3 von Willebrand disease: assessment of complications and approaches to treatment—results of a patient and Hemophilia Treatment Center Survey in the United States. Haemophilia 2004 Jul;10(4):360-366.
  213. Kaufmann JE, Vischer UM. Cellular mechanisms of the hemostatic effects of desmopressin (DDAVP). J Thromb Haemost 2003 Apr;1(4):682-689.
  214. Mannucci PM. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years. Blood 1997 Oct;90(7):2515-2521.
  215. Mannucci PM. Treatment of von Willebrand's disease. N Engl J Med 2004 Aug;351(7):683-694.
  216. Kobrinsky NL, Doyle JJ, Israels ED, Winter JS, Cheang MS, Walker RD, Bishop AJ. Absent factor VIII response to synthetic vasopressin analogue (DDAVP) in nephrogenic diabetes insipidus. Lancet 1985 Jun;1(8441):1293-1294.
  217. Kaufmann JE, Oksche A, Wollheim CB, Gunther G, Rosenthal W, Vischer UM. Vasopressin-induced von Willebrand factor secretion from endothelial cells involves V2 receptors and cAMP. J Clin Invest 2000 Jul;106(1):107-116.
  218. Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A. 1-Deamino-8-d-arginine vasopressin: a new pharmacological approach to the management of haemophilia and von Willebrand's disease. Lancet 1977 Apr;1(8017):869-872.
  219. Cash JD, Gader AM, da Costa J. Proceedings: the release of plasminogen activator and factor VIII to lysine vasopressin, arginine vasopressin, 1-desamino-8-d-arginine vasopressin, angiotensin and oxytocin in man. Br J Haematol 1974 Jun;27(2):363-364.
  220. Mannucci PM, Åberg M, Nilsson IM, Robertson B. Mechanism of plasminogen activator and factor VIII increase after vasoactive drugs. Br J Haematol 1975 May;30(1):81-93.
  221. Castaman G, Lattuada A, Mannucci PM, Rodeghiero F. Factor VIII:C increases after desmopressin in a subgroup of patients with autosomal recessive severe von Willebrand disease. Br J Haematol 1995 Jan;89(1):147-151.
  222. Castaman G, Rodeghiero F. Desmopressin and type II B von Willebrand disease. Haemophilia 1996 Apr;2(2):73-77.
  223. de la Fuente B, Kasper CK, Rickles FR, Hoyer LW. Response of patients with mild and moderate hemophilia A and von Willebrand's disease to treatment with desmopressin. Ann Intern Med 1985 Jul;103(1):6-14.
  224. Federici AB, Mazurier C, Berntorp E, Lee CA, Scharrer I, Goudemand J, Lethagen S, Nitu I, Ludwig G, Hilbert L, et al. Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study. Blood 2004 Mar;103(6):2032-2038.
  225. Lethagen S, Harris AS, Sjörin E, Nilsson IM. Intranasal and intravenous administration of desmopressin: effect on FVIII/vWF, pharmacokinetics and reproducibility. Thromb Haemost 1987 Dec;58(4):1033-1036.
  226. Mannucci PM, Bettega D, Cattaneo M. Patterns of development of tachyphylaxis in patients with haemophilia and von Willebrand disease after repeated doses of desmopressin (DDAVP). Br J Haematol 1992 Sep;82(1):87-93.
  227. Mannucci PM, Canciani MT, Rota L, Donovan BS. Response of factor VIII/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand's disease. Br J Haematol 1981 Feb;47(2):283-293.
  228. Mazurier C, Gaucher C, Jorieux S, Goudemand M. Biological effect of desmopressin in eight patients with type 2N ('Normandy') von Willebrand disease. Collaborative Group. Br J Haematol 1994 Dec;88(4):849-854.
  229. McKeown LP, Connaghan G, Wilson O, Hansmann K, Merryman P, Gralnick HR. 1-Desamino-8-arginine-vasopressin corrects the hemostatic defects in type 2B von Willebrand's disease. Am J Hematol 1996 Feb;51(2):158-163.
  230. Revel-Vilk S, Schmugge M, Carcao MD, Blanchette P, Rand ML, Blanchette VS. Desmopressin (DDAVP) responsiveness in children with von Willebrand disease. J Pediatr Hematol Oncol 2003 Nov;25(11):874-879.
  231. Rodeghiero F, Castaman G, Di Bona E, Ruggeri M. Consistency of responses to repeated DDAVP infusions in patients with von Willebrand's disease and hemophilia A. Blood 1989 Nov;74(6):1997-2000.
  232. Rodeghiero F, Castaman G, Di Bona E, Ruggeri M, Lombardi R, Mannucci PM. Hyper-responsiveness to DDAVP for patients with type I von Willebrand's disease and normal intra-platelet von Willebrand factor. Eur J Haematol 1988 Feb;40(2):163-167.
  233. Mannucci PM, Vicente V, Alberca I, Sacchi E, Longo G, Harris AS, Lindquist A. Intravenous and subcutaneous administration of desmopressin (DDAVP) to hemophiliacs: pharmacokinetics and factor VIII responses. Thromb Haemost 1987 Dec;58(4):1037-1039.
  234. Bond L, Bevan D. Myocardial infarction in a patient with hemophilia treated with DDAVP. N Engl J Med 1988 Jan;318(2):121.
  235. Byrnes JJ, Larcada A, Moake JL. Thrombosis following desmopressin for uremic bleeding. Am J Hematol 1988 May;28(1):63-65.
  236. Virtanen R, Kauppila M, Itälä M. Percutaneous coronary intervention with stenting in a patient with haemophilia A and an acute myocardial infarction following a single dose of desmopressin. Thromb Haemost 2004 Nov; 92(5):1154-1156.
  237. Ruggeri ZM, Mannucci PM, Lombardi R, Federici AB, Zimmerman TS. Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes. Blood 1982 Jun;59(6):1272-1278.
  238. van Schooten CJ, Tjernberg P, Westein E, Terraube V, Castaman G, van Mourik JA, Hollestelle MJ, Vos HL, Bertina RM, van den Berg HM, et al. Cysteine-mutations in von Willebrand factor associated with increased clearance. J Thromb Haemost 2005 Oct;3(10): 2228-2237.
  239. Casonato A, Steffan A, Pontara E, Zucchetto A, Rossi C, De Marco L, Girolami A. Post-DDAVP thrombocytopenia in type 2B von Willebrand disease is not associated with platelet consumption: failure to demonstrate glycocalicin increase or platelet activation. Thromb Haemost 1999 Feb;81(2):224-228.
  240. Allen GC, Armfield DR, Bontempo FA, Kingsley LA, Goldstein NA, Post JC. Adenotonsillectomy in children with von Willebrand disease. Arch Otolaryngol Head Neck Surg 1999 May; 125(5):547-551.
  241. Derkay CS, Werner E, Plotnick E. Management of children with von Willebrand disease undergoing adenotonsillectomy. Am J Otolaryngol 1996 May;17(3):172-177.
  242. Federici AB, Sacco R, Stabile F, Carpenedo M, Zingaro E, Mannucci PM. Optimising local therapy during oral surgery in patients with von Willebrand disease: effective results from a retrospective analysis of 63 cases. Haemophilia 2000 Mar;6(2):71-77.
  243. Jimenez-Yuste V, Prim MP, De Diego JI, Villar A, Quintana M, Rabanal I, Sastre N, Hernandez- Navarro F. Otolaryngologic surgery in children with von Willebrand disease. Arch Otolaryngol Head Neck Surg 2002 Dec;128(12):1365-1368.
  244. Kreuz W, Mentzer D, Becker S, Scharrer I, Kornhuber B. Haemate-P® in children with von Willebrand's disease. Haemostasis 1994 Sep; 24(5):304-310.
  245. Manno CS, Butler RB, Farace L, Cohen AR. Successful management of patients with type 1 von Willebrand's disease with desmopressin acetate for tonsillectomy (abstract). Haemophilia 1998;4:288.
  246. Mariana G, Ciavarella N, Mazzucconi MG, Antoncecchi S, Solinas S, Ranieri P, Pettini P, Agrestini F, Mandelli F. Evaluation of the effectiveness of DDAVP in surgery and in bleeding episodes in haemophilia and von Willebrand's disease. A study on 43 patients. Clin Lab Haematol 1984;6(3):229-238.
  247. Nitu-Whalley IC, Griffioen A, Harrington C, Lee CA. Retrospective review of the management of elective surgery with desmopressin and clotting factor concentrates in patients with von Willebrand disease. Am J Hematol 2001 Apr;66(4):280-284.
  248. Saulnier J, Marey A, Horellou MH, Goudemand J, Lepoutre F, Donazzan M, Gazengel C, Torchet M, Letang C, Schuhmann C, et al. Evaluation of desmopressin for dental extractions in patients with hemostatic disorders. Oral Surg Oral Med Oral Pathol 1994 Jan;77(1):6-12.
  249. Shah SB, Lalwani AK, Koerper MA. Perioperative management of von Willebrand's disease in otolaryngologic surgery. Laryngoscope 1998 Jan;108(1 Pt 1):32-36.
  250. Theiss W, Schmidt G. DDAVP in von Willebrand's disease: repeated administration and the behaviour of the bleeding time. Thromb Res 1978 Dec;13(6):1119-1123.
  251. Dobrkovska A, Krzensk U, Chediak JR. Pharmacokinetics, efficacy and safety of Humate-P® in von Willebrand disease. Haemophilia 1998;(4 Suppl 3):33-39.
  252. Hanna WT, Bona RD, Zimmerman CE, Carta CA, Hebert GZ, Rickles FR. The use of intermediate and high purity factor VIII products in the treatment of von Willebrand disease. Thromb Haemost 1994 Feb;71(2):173-179.
  253. Lillicrap D, Poon MC, Walker I, Xie F, Schwartz BA. Efficacy and safety of the factor VIII/von Willebrand factor concentrate, Haemate-P/Humate-P: ristocetin cofactor unit dosing in patients with von Willebrand disease. Thromb Haemost 2002 Feb;87(2):224-230.
  254. Lubetsky A, Schulman S, Varon D, Martinowitz U, Kenet G, Gitel S, Inbal A. Safety and efficacy of continuous infusion of a combined factor VIII-von Willebrand factor (vWF) concentrate (Haemate-P®) in patients with von Willebrand disease. Thromb Haemost 1999 Feb;81(2): 229-233.
  255. Michiels JJ, Berneman ZN, van der Planken M, Schroyens W, Budde U, van Vliet HH. Bleeding prophylaxis for major surgery in patients with type 2 von Willebrand disease with an intermediate purity factor VIII-von Willebrand factor concentrate (Haemate-P). Blood Coagul Fibrinolysis 2004 Jun;15(4):323-330.
  256. Thompson AR, Gill JC, Ewenstein BM, Mueller-Velten G, Schwartz BA, Humate-P® Study Group. Successful treatment for patients with von Willebrand disease undergoing urgent surgery using factor VIII/VWF concentrate (Humate-P®). Haemophilia 2004 Jan;10(1):42-51.
  257. Holmberg L, Nilsson IM, Borge L, Gunnarsson M, Sjorin E. Platelet aggregation induced by 1-desamino-8-D-arginine vasopressin (DDAVP) in type IIB von Willebrand's disease. N Engl J Med 1983 Oct;309(14):816-821.
  258. Fowler WE, Berkowitz LR, Roberts HR. DDAVP for type IIB von Willebrand disease. Blood 1989 Oct;74(5):1859-1860.
  259. Casonato A, Pontara E, Dannhaeuser D, Bertomoro A, Sartori MT, Zerbinati P, Girolami A. Re-evaluation of the therapeutic efficacy of DDAVP in type IIB von Willebrand's disease. Blood Coagul Fibrinolysis 1994 Dec;5(6):959-964.
  260. Piot B, Sigaud-Fiks M, Huet P, Fressinaud E, Trossaert M, Mercier J. Management of dental extractions in patients with bleeding disorders. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002 Mar;93(3):247-250.
  261. Sutor AH. DDAVP is not a panacea for children with bleeding disorders. Br J Haematol 2000 Feb;108(2):217-227.
  262. Dunn AL, Powers JR, Ribeiro MJ, Rickles FR, Abshire TC. Adverse events during use of intranasal desmopressin acetate for haemophilia A and von Willebrand disease: a case report and review of 40 patients. Haemophilia 2000 Jan;6(1):11-14.
  263. Lethagen S, Frick K, Sterner G. Antidiuretic effect of desmopressin given in hemostatic dosages to healthy volunteers. Am J Hematol 1998 Feb;57(2):153-159.
  264. Bertholini DM, Butler CS. Severe hyponatraemia secondary to desmopressin therapy in von Willebrand's disease. Anaesth Intensive Care 2000 Apr;28(2):199-201.
  265. Das P, Carcao M, Hitzler J. DDAVP-induced hyponatremia in young children. J Pediatr Hematol Oncol 2005 Jun;27(6):330-332.
  266. Smith TJ, Gill JC, Ambruso DR, Hathaway WE. Hyponatremia and seizures in young children given DDAVP. Am J Hematol 1989 Jul;31(3):199-202.
  267. McLeod BC. Myocardial infarction in a blood donor after administration of desmopressin. Lancet 1990 Nov;336(8723):1137-1138.
  268. Lowe GD. Desmopressin and myocardial infarction. Lancet 1989 Apr;1(8643):895-896.
  269. Grunwald Z, Sather SD. Intraoperative cerebral infarction after desmopressin administration in infant with end-stage renal disease. Lancet 1995 May;345(8961):1364-1365.
  270. Levi M, Cromheecke ME, de Jonge E, Prins MH, de Mol BJ, Briët E, Büller HR. Pharmacological strategies to decrease excessive blood loss in cardiac surgery: a meta-analysis of clinically relevant endpoints. Lancet 1999 Dec;354(9194):1940-1947.
  271. Kouides PA. Females with von Willebrand disease: 72 years as the silent majority. Haemophilia 1998 Jul;4(4):665-676.
  272. Kujovich JL. von Willebrand disease and pregnancy. J Thromb Haemost 2005 Feb;3(2):246-253.
  273. Mannucci PM. Use of desmopressin (DDAVP) during early pregnancy in factor VIII-deficient women. Blood 2005 Apr;105(8):3382.
  274. Ray JG. DDAVP use during pregnancy: an analysis of its safety for mother and child. Obstet Gynecol Surv 1998 Jul;53(7):450-455.
  275. Agrawal YP, Dzik W. The vWF content of factor VIII concentrates. Transfusion 2001 Jan;41(1):153-154.
  276. Chang AC, Rick ME, Ross PL, Weinstein MJ. Summary of a workshop on potency and dosage of von Willebrand factor concentrates. Haemophilia 1998;4 (Suppl 3):1-6.
  277. Mannucci PM, Lattuada A, Ruggeri ZM. Proteolysis of von Willebrand factor in therapeutic plasma concentrates. Blood 1994;83(10):3018-3027.
  278. Morfini M, Mannucci PM, Tenconi PM, Longo G, Mazzucconi MG, Rodeghiero F, Ciavarella N, De Rosa V, Arter A. Pharmacokinetics of monoclonally-purified and recombinant factor VIII in patients with severe von Willebrand disease. Thromb Haemost 1993 Aug;70(2):270-272.
  279. Metzner HJ, Hermentin P, Cuesta-Linker T, Langner S, Muller HG, Friedebold J. Characterization of factor VIII/von Willebrand factor concentrates using a modified method of von Willebrand factor multimer analysis. Haemophilia 1998;4 (Suppl 3):25-32.
  280. Makris M, Colvin B, Gupta V, Shields ML, Smith MP. Venous thrombosis following the use of intermediate purity FVIII concentrate to treat patients with von Willebrand's disease. Thromb Haemost 2002 Sep;88(3):387-388.
  281. Mannucci PM. Venous thromboembolism in von Willebrand disease. Thromb Haemost 2002 Sep;88(3):378-379.
  282. Mannucci PM, Chediak J, Hanna W, Byrnes J, Ledford M, Ewenstein BM, Retzios AD, Kapelan BA, Schwartz RS, Kessler C, et al. Treatment of von Willebrand disease with a high-purity factor VIII/von Willebrand factor concentrate: a prospective, multicenter study. Blood 2002 Jan;99(2):450-456.
  283. Srivastava A. von Willebrand disease in the developing world. Semin Hematol 2005 Jan;42(1):36-41.
  284. Budde U, Metzner HJ, Muller HG. Comparative analysis and classification of von Willebrand factor/factor VIII concentrates: impact on treatment of patients with von Willebrand disease. Semin Thromb Hemost 2006 Sep;32(6):626-635.
  285. Lethagen S, Carlson M, Hillarp A. A comparative in vitro evaluation of six von Willebrand factor concentrates. Haemophilia 2004 May;10(3):243-249.
  286. FDA Additional Standards for Human Blood and Blood Products, 21 C.F.R. §640.54(b)(1)(2006).
  287. Pomper GJ, Wu Y, Snyder EL. Risks of transfusion-transmitted infections: 2003. Curr Opin Hematol 2003 Nov;10(6):412-418.
  288. National Hemophilia Foundation (NHF). 2003. MASAC Bulletin 151. MASAC recommendations concerning the treatment of hemophilia and other bleeding disorders. Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=203.
  289. Evatt BL, Austin H, Leon G, Ruiz-Saez A, De Bosch N. Haemophilia therapy: assessing the cumulative risk of HIV exposure by cryoprecipitate. Haemophilia 1999 Sep;5(5):295-300.
  290. Scharrer I, Vigh T, Aygoren-Pürsün E. Experience with Haemate-P in von Willebrand's disease in adults. Haemostasis 1994 Sep;24(5):298-303.
  291. Gill JC, Ewenstein BM, Thompson AR, Mueller-Velten G, Schwartz BA, Humate-P® Study Group. Successful treatment of urgent bleeding in von Willebrand disease with factor VIII/VWF concentrate (Humate-P®): use of the ristocetin cofactor assay (VWF:RCo) to measure potency and to guide therapy. Haemophilia 2003 Nov;9(6):688-695.
  292. Turecek PL, Gritsch H, Richter G, Auer W, Pichler L, Schwarz HP. Assessment of bleeding for the evaluation of therapeutic preparations in small animal models of antibody-induced hemophilia and von Willebrand disease. Thromb Haemost 1997 Mar;77(3):591-599.
  293. Castillo R, Escolar G, Monteagudo J, Aznar-Salatti J, Reverter JC, Ordinas A. Hemostasis in patients with severe von Willebrand disease improves after normal platelet transfusion and normalizes with further correction of the plasma defect. Transfusion 1997 Aug;37(8):785-790.
  294. Castillo R, Monteagudo J, Escolar G, Ordinas A, Magallon M, Martin Villar J. Hemostatic effect of normal platelet transfusion in severe von Willebrand disease patients. Blood 1991 May;77(9):1901-1905.
  295. Miller RA, May MW, Hendry WF, Whitfield HN, Wickham JE. The prevention of secondary haemorrhage after prostatectomy: the value of antifibrinolytic therapy. Br J Urol 1980 Feb;52(1):26-28.
  296. Mannucci PM. Hemostatic drugs. N Engl J Med 1998 Jul;339(4):245-253.
  297. Rakocz M, Mazar A, Varon D, Spierer S, Blinder D, Martinowitz U. Dental extractions in patients with bleeding disorders. The use of fibrin glue. Oral Surg Oral Med Oral Pathol 1993 Mar;75(3):280-282.
  298. Zwischenberger JB, Brunston RL Jr, Swann JR, Conti VR. Comparison of two topical collagen-based hemostatic sponges during cardiothoracic procedures. J Invest Surg 1999 Mar;12(2):101-106.
  299. National Hemophilia Foundation (NHF). 2001. MASAC Recommendation 128. Recommendations for hepatitis A and B immunization of individuals with bleeding disorders. Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=240.
  300. Barbui T, Rodeghiero F, Dini E. The aspirin tolerance test in von Willebrand's disease. Thromb Haemost 1977 Aug;38(2):510-513.
  301. Rosenstein R, Zacharski LR. The diagnostic value of the aspirin tolerance test. Thromb Res 1979;16(1-2):219-230.
  302. Stuart MJ, Miller ML, Davey FR, Wolk JA. The post-aspirin bleeding time: a screening test for evaluating haemostatic disorders. Br J Haematol 1979 Dec;43(4):649-659.
  303. 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 Oct;92(8):2707-2711.
  304. Arkel YS, Lynch J, Kamiyama M. Treatment of acquired von Willebrand syndrome with intravenous immunoglobulin. Thromb Haemost 1994 Oct;72(4):643-644.
  305. Macik BG, Gabriel DA, White GC 2nd, High K, Roberts H. The use of high-dose intravenous gamma-globulin in acquired von Willebrand syndrome. Arch Pathol Lab Med 1988 Feb;112(2):143-146.
  306. van Genderen PJ, Terpstra W, Michiels JJ, Kapteijn L, van Vliet HH. High-dose intravenous immunoglobulin delays clearance of von Willebrand factor in acquired von Willebrand disease. Thromb Haemost 1995 May;73(5):891-892.
  307. Basso IN, Keeling D. Myocardial infarction following recombinant activated factor VII in a patient with type 2A von Willebrand disease. Blood Coagul Fibrinolysis 2004 Sep;15(6):503-504.
  308. Weinstein M, Ware JA, Troll J, Salzman E. Changes in von Willebrand factor during cardiac surgery: effect of desmopressin acetate. Blood 1988 Jun;71(6):1648-1655.
  309. Rahmani R, Rozen P, Papo J, Iellin A, Seligsohn U. Association of von Willebrand's disease with plasma cell dyscrasia and gastrointestinal angiodysplasia. Isr J Med Sci 1990 Sep;26(9):504-509.
  310. Veyradier A, Balian A, Wolf M, Giraud V, Montembault S, Obert B, Dagher I, Chaput JC, Meyer D, Naveau S. Abnormal von Willebrand factor in bleeding angiodysplasias of the digestive tract. Gastroenterology 2001 Feb;120(2):346-353.
  311. Morris ES, Hampton KK, Nesbitt IM, Preston FE, Thomas EG, Makris M. The management of von Willebrand's disease-associated gastrointestinal angiodysplasia. Blood Coagul Fibrinolysis 2001 Mar;12(2):143-148.
  312. Baumgartner-Parzer SM, Wagner L, Reining G, Sexl V, Nowotny P, Muller M, Brunner M, Waldhausl W. Increase by tri-iodothyronine of endothelin-1, fibronectin and von Willebrand factor in cultured endothelial cells. J Endocrinol 1997 Aug;154(2):231-239.
  313. Michiels JJ, Schroyens W, Berneman Z, van der Planken M. Acquired von Willebrand syndrome type 1 in hypothyroidism: reversal after treatment with thyroxine. Clin Appl Thromb Hemost 2001 Apr;7(2):113-115.
  314. Kirtava A, Crudder S, Dilley A, Lally C, Evatt B. Trends in clinical management of women with von Willebrand disease: a survey of 75 women enrolled in haemophilia treatment centres in the United States. Haemophilia 2004 Mar;10(2):158-161.
  315. Ragni MV, Bontempo FA, Hassett AC. von Willebrand disease and bleeding in women. Haemophilia 1999 Sep;5(5):313-317.
  316. Chuong CJ, Brenner PF. Management of abnormal uterine bleeding. Am J Obstet Gynecol 1996 Sep;175(3 Pt 2):787-792.
  317. Kadir RA, Lee CA, Sabin CA, Pollard D, Economides DL. DDAVP nasal spray for treatment of menorrhagia in women with inherited bleeding disorders: a randomized placebo-controlled crossover study. Haemophilia 2002 Nov;8(6):787-793.
  318. James A, Matchar DB, Myers ER. Testing for von Willebrand disease in women with menorrhagia: a systematic review. Obstet Gynecol 2004 Aug;104(2):381-388.
  319. Schafer AI. Effects of nonsteroidal antiinflammatory drugs on platelet function and systemic hemostasis. J Clin Pharmacol 1995 Mar;35(3):209-219.
  320. Amesse LS, Pfaff-Amesse T, Leonardi R, Uddin D, French JA 2nd. Oral contraceptives and DDAVP nasal spray: patterns of use in managing vWD-associated menorrhagia: a single-institution study. J Pediatr Hematol Oncol 2005 Jul;27(7):357-363.
  321. Kouides PA, Phatak PD, Burkart P, Braggins C, Cox C, Bernstein Z, Belling L, Holmberg P, MacLaughlin W, Howard F. Gynaecological and obstetrical morbidity in women with type I von Willebrand disease: results of a patient survey. Haemophilia 2000 Nov;6(6):643-648.
  322. Foster PA. The reproductive health of women with von Willebrand Disease unresponsive to DDAVP: results of an international survey. On behalf of the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the ISTH. Thromb Haemost 1995 Aug;74(2):784-790.
  323. Greer IA, Lowe GD, Walker JJ, Forbes CD. Haemorrhagic problems in obstetrics and gynaecology in patients with congenital coagulopathies. Br J Obstet Gynaecol 1991 Sep;98(9):909-918.
  324. Kingman CE, Kadir RA, Lee CA, Economides DL. The use of levonorgestrel-releasing intrauterine system for treatment of menorrhagia in women with inherited bleeding disorders. BJOG 2004 Dec;111(12):1425-1428.
  325. Leissinger C, Becton D, Cornell C, Jr., Cox Gill J. High-dose DDAVP intranasal spray (Stimate) for the prevention and treatment of bleeding in patients with mild haemophilia A, mild or moderate type 1 von Willebrand disease and symptomatic carriers of haemophilia A. Haemophilia 2001 May;7(3):258-266.
  326. Rodeghiero F, Castaman G, Mannucci PM. Prospective multicenter study on subcutaneous concentrated desmopressin for home treatment of patients with von Willebrand disease and mild or moderate hemophilia A. Thromb Haemost 1996 Nov;76(5):692-696.
  327. Mohri H. High dose of tranexamic acid for treatment of severe menorrhagia in patients with von Willebrand disease. J Thromb Thrombolysis 2002 Dec;14(3):255-257.
  328. Petitti DB. Clinical practice. Combination estrogen-progestin oral contraceptives. N Engl J Med 2003 Oct;349(15):1443-1450.
  329. Rosendaal FR, Helmerhorst FM, Vandenbroucke JP. Female hormones and thrombosis. Arterioscler Thromb Vasc Biol 2002 Feb;22(2):201-210.
  330. Beller FK, Ebert C. Effects of oral contraceptives on blood coagulation. A review. Obstet Gynecol Surv 1985 Jul;40(7):425-436.
  331. Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Variations in coagulation factors in women: effects of age, ethnicity, menstrual cycle and combined oral contraceptive. Thromb Haemost 1999 Nov;82(5):1456-1461.
  332. Middeldorp S, Meijers JC, van den Ende AE, van Enk A, Bouma BN, Tans G, Rosing J, Prins MH, Büller HR. Effects on coagulation of levonorgestrel- and desogestrel-containing low dose oral contraceptives: a cross-over study. Thromb Haemost 2000 Jul;84(1):4-8.
  333. Nilsson L, Rybo G. Treatment of menorrhagia. Am J Obstet Gynecol 1971 Jul;110(5):713-720.
  334. Milman N, Clausen J, Byg KE. Iron status in 268 Danish women aged 18-30 years: influence of menstruation, contraceptive method, and iron supplementation. Ann Hematol 1998 Jul; 77(1-2):13-19.
  335. Rivera R, Almonte H, Arreola M, Lopez F, Monarrez G, Navarro C, Ortiz E, Perkin GW, Ruiz R. The effects of three different regimens of oral contraceptives and three different intrauterine devices on the levels of hemoglobin, serum iron and iron binding capacity in anemic women. Contraception 1983 Mar;27(3):311-327.
  336. WHO Task Force. Effects of contraceptives on hemoglobin and ferritin. Contraception 1998 Nov;58(5):262-273.
  337. Grimes DA. The safety of oral contraceptives: epidemiologic insights from the first 30 years. Am J Obstet Gynecol 1992 Jun;166(6 Pt 2): 1950-1954.
  338. Sicat BL. Ortho Evra, a new contraceptive patch. Pharmacotherapy 2003 Apr;23(4):472-480.
  339. Davis A, Godwin A, Lippman J, Olson W, Kafrissen M. Triphasic norgestimate-ethinyl estradiol for treating dysfunctional uterine bleeding. Obstet Gynecol 2000 Dec;96(6):913-920.
  340. Kadir RA, Economides DL, Sabin CA, Pollard D, Lee CA. Assessment of menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders. Haemophilia 1999 Jan;5(1):40-48.
  341. Rubin G, Wortman M, Kouides PA. Endometrial ablation for von Willebrand disease-related menorrhagia-experience with seven cases. Haemophilia 2004 Sep;10(5):477-482.
  342. Bain C, Cooper KG, Parkin DE. Microwave endometrial ablation versus endometrial resection: a randomized controlled trial. Obstet Gynecol 2002 Jun;99(6):983-987.
  343. Boujida VH, Philipsen T, Pelle J, Joergensen JC. Five-year follow-up of endometrial ablation: endometrial coagulation versus endometrial resection. Obstet Gynecol 2002 Jun;99(6):988-992.
  344. Comino R, Torrejon R. Hysterectomy after endometrial ablation-resection. J Am Assoc Gynecol Laparosc 2004 Nov;11(4):495-499.
  345. Dutton C, Ackerson L, Phelps-Sandall B. Outcomes after rollerball endometrial ablation for menorrhagia. Obstet Gynecol 2001 Jul;98(1):35-39.
  346. Phillips G, Chien PF, Garry R. Risk of hysterectomy after 1000 consecutive endometrial laser ablations. Br J Obstet Gynaecol 1998 Aug;105(8):897-903.
  347. Kuppermann M, Varner RE, Summitt RL Jr, Learman LA, Ireland C, Vittinghoff E, Stewart AL, Lin F, Richter HE, Showstack J, et al. Effect of hysterectomy vs medical treatment on health-related quality of life and sexual functioning: the medicine or surgery (Ms) randomized trial. JAMA 2004 Mar;291(12):1447-1455.
  348. Unger JB, Meeks GR. Hysterectomy after endometrial ablation. Am J Obstet Gynecol 1996 Dec;175(6):1432-1436.
  349. Bottini E, Pareti FI, Mari D, Mannucci PM, Muggiasca ML, Conti M. Prevention of hemoperitoneum during ovulation by oral contraceptives in women with type III von Willebrand disease and afibrinogenemia. Case reports. Haematologica 1991 Sep;76(5):431-433.
  350. Ghosh K, Mohanty D, Pathare AV, Jijina F. Recurrent haemoperitoneum in a female patient with type III von Willebrand's disease responded to administration of oral contraceptive. Haemophilia 1998 Sep;4(5):767-768.
  351. Gomez A, Lucia JF, Perella M, Aguilar C. Haemoperitoneum caused by haemorrhagic corpus luteum in a patient with type 3 von Willebrand's disease. Haemophilia 1998 Jan;4(1):60-62.
  352. Jarvis RR, Olsen ME. Type I von Willebrand's disease presenting as recurrent corpus hemorrhagicum. Obstet Gynecol 2002 May;99(5 Pt 2):887-888.
  353. Meschengieser SS, Alberto MF, Salviu J, Bermejo E, Lazzari MA. Recurrent haemoperitoneum in a mild von Willebrand's disease combined with a storage pool deficit. Blood Coagul Fibrinolysis 2001 Apr;12(4):207-209.
  354. Kadir RA, Lee CA, Sabin CA, Pollard D, Economides DL. Pregnancy in women with von Willebrand's disease or factor XI deficiency. Br J Obstet Gynaecol 1998 Mar;105(3):314-321.
  355. Kadir RA. Women and inherited bleeding disorders: pregnancy and delivery. Semin Hematol 1999 Jul;36(3 Suppl 4):28-35.
  356. Kouides PA. Obstetric and gynaecological aspects of von Willebrand disease. Best Pract Res Clin Haematol 2001 Jun;14(2):381-399.
  357. Mannucci PM. How I treat patients with von Willebrand disease. Blood 2001 Apr;97(7):1915-1919.
  358. Kullander S, Nilsson IM. Human placental transfer of an antifibrinolytic agent (AMCA). Acta Obstet Gynecol Scand 1970;49(3):241-242.
  359. Astedt B, Nilsson IM. Recurrent abruptio placentae treated with the fibrinolytic inhibitor tranexamic acid. Br Med J 1978 Mar;1(6115):756-757.
  360. Lindoff C, Rybo G, Astedt B. Treatment with tranexamic acid during pregnancy, and the risk of thrombo-embolic complications. Thromb Haemost 1993 Aug;70(2):238-240.
  361. Storm O, Weber J. Prolonged treatment with tranexamic acid (Cyclocapron) during pregnancy. Ugeskr Laeger 1976 Jul;138(29):1781-1782. (Dan).
  362. Sundqvist SB, Nilsson IM, Svanberg L, Cronberg S. Pregnancy and parturition in a patient with severe Glanzmann's thrombasthenia. Scand J Haematol 1981 Sep;27(3):159-164.
  363. Svanberg L, Astedt B, Nilsson IM. Abruptio placentae—treatment with the fibrinolytic inhibitor tranexamic acid. Acta Obstet Gynecol Scand 1980;59(2):127-130.
  364. Walzman M, Bonnar J. Effects of tranexamic acid on the coagulation and fibrinolytic systems in pregnancy complicated by placental bleeding. Arch Toxicol 1982;(Suppl 5):214-220.
  365. Howorka E, Olasinski R, Wyrzykiewicz T. Effect of EACA administered to female rabbits during pregnancy on the fetuses. Patol Pol 1970 Jul; 21(3):311-314. (Pol).
  366. Kassam SH, Hadi HA, Fadel HE, Sanchez-Ramos L, Milner PF. Sickle-cell-induced hematuria in pregnancy. The current diagnostic and therapeutic approach. J Reprod Med 1984 Feb; 29(2):117-121.
  367. Everett C. Incidence and outcome of bleeding before the 20th week of pregnancy: prospective study from general practice. BMJ 1997 Jul;315(7099):32-34.
  368. Nybo Andersen AM, Wohlfahrt J, Christens P, Olsen J, Melbye M. Maternal age and fetal loss: population based register linkage study. BMJ 2000 Jun;320(7251):1708-1712.
  369. Burlingame J, McGaraghan A, Kilpatrick S, Hambleton J, Main E, Laros RK. Maternal and fetal outcomes in pregnancies affected by von Willebrand disease type 2. Am J Obstet Gynecol 2001 Jan;184(2):229-230.
  370. Caliezi C, Tsakiris DA, Behringer H, Kuhne T, Marbet GA. Two consecutive pregnancies and deliveries in a patient with von Willebrand's disease type 3. Haemophilia 1998 Nov;4(6):845-849.
  371. Chediak JR, Alban GM, Maxey B. von Willebrand's disease and pregnancy: management during delivery and outcome of offspring. Am J Obstet Gynecol 1986 Sep;155(3):618-624.
  372. Stedeford JC, Pittman JA. Von Willebrand's disease and neuroaxial anaesthesia. Anaesthesia 2000 Dec;55(12):1228-1229.
  373. Ross MG, Ervin MG, Novak D. Placental and fetal physiology. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Obstetrics: normal and problem pregnancies. 4th ed. New York: Churchill Livingstone; 2002. 37-62.
  374. Benedetti TJ. Obstetric hemorrhage. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Obstetrics: normal and problem pregnancies. 5th ed. New York: Churchill Livingstone; 2005. 503-538.
  375. Lee CA, Chi C, Pavord SR, Bolton-Maggs PHB, Pollard D, Hinchcliffe-Wood A, Kadir RA. The obstetric and gynaecological management of women with inherited bleeding disorders—review with guidelines produced by a taskforce of UK Haemophilia Doctors' Organization. Haemophilia 2006 Jul;12(4):301-336.
  376. Ramsahoye BH, Davies SV, Dasani H, Pearson JF. Obstetric management in von Willebrand's disease: a report of 24 pregnancies and a review of the literature. Haemophilia 1995 Apr;1(2):140-144.
  377. Conti M, Mari D, Conti E, Muggiasca ML, Mannucci PM. Pregnancy in women with different types of von Willebrand disease. Obstet Gynecol 1986 Aug;68(2):282-285.
  378. Gardella C, Taylor M, Benedetti T, Hitti J, Critchlow C. The effect of sequential use of vacuum and forceps for assisted vaginal delivery on neonatal and maternal outcomes. Am J Obstet Gynecol 2001 Oct;185(4):896-902.
  379. Edwards A, Ellwood DA. Ultrasonographic evaluation of the postpartum uterus. Ultrasound Obstet Gynecol 2000 Dec;16(7):640-643.
  380. Marchant S, Alexander J, Garcia J, Ashurst H, Alderdice F, Keene J. A survey of women's experiences of vaginal loss from 24 hours to three months after childbirth (the BLiPP study). Midwifery 1999 Jun;15(2):72-81.
  381. Visness CM, Kennedy KI, Ramos R. The duration and character of postpartum bleeding among breast-feeding women. Obstet Gynecol 1997 Feb;89(2):159-163.
  382. Dahlman T, Hellgren M, Blomback M. Changes in blood coagulation and fibrinolysis in the normal puerperium. Gynecol Obstet Invest 1985;20(1):37-44.
  383. Sánchez-Luceros A, Meschengieser SS, Marchese C, Votta R, Casais P, Woods AI, Nadal MV, Salviú MJ, Lazzari MA. Factor VIII and von Willebrand factor changes during normal pregnancy and puerperium. Blood Coagul Fibrinolysis 2003 Oct;14(7):647-651.
  384. Hoveyda F, MacKenzie IZ. Secondary postpartum haemorrhage: incidence, morbidity and current management. BJOG 2001 Sep;108(9):927-930.
  385. Lee CY, Madrazo B, Drukker BH. Ultrasonic evaluation of the postpartum uterus in the management of postpartum bleeding. Obstet Gynecol 1981 Aug;58(2):227-232.
  386. Roqué H, Funai E, Lockwood CJ. von Willebrand disease and pregnancy. J Matern Fetal Med 2000 Sep;9(5):257-266.
  387. Weiss HJ. The bleeding tendency in patients with low von Willebrand factor and type 1 phenotype is greater in the presence of impaired collagen-induced platelet aggregation. J Thromb Haemost 2004 Jan;2(1):198-199.
  388. Kunicki TJ, Federici AB, Salomon DR, Koziol JA, Head SR, Mondala TS, Chismar JD, Baronciani L, Canciani MT, Peake IR. An association of candidate gene haplotypes and bleeding severity in von Willebrand disease (VWD) type 1 pedigrees. Blood 2004 Oct;104(8):2359-2367.
  389. Kyrle PA, Niessner H, Dent J, Panzer S, Brenner B, Zimmerman TS, Lechner K. IIB von Willebrand's disease: pathogenetic and therapeutic studies. Br J Haematol 1988 May;69(1):55-59.
  390. Mauz-Körholz C, Budde U, Kruck H, Korholz D, Gobel U. Management of severe chronic thrombocytopenia in von Willebrand's disease type 2B. Arch Dis Child 1998 Mar;78(3):257-260.
  391. Ruggeri ZM, Lombardi R, Gatti L, Bader R, Valsecchi C, Zimmerman TS. Type IIB von Willebrand’s disease: differential clearance of endogenous versus transfused large multimer von Willebrand factor. Blood 1982 Dec;60(6):1453-1456.
  392. Lopez-Fernandez MF, Blanco-Lopez MJ, Castineira MP, Batlle J. Further evidence for recessive inheritance of von Willebrand disease with abnormal binding of von Willebrand factor to factor VIII. Am J Hematol 1992 May;40(1):20-27.
  393. Goudemand J, Negrier C, Ounnoughene N, Sultan Y. Clinical management of patients with von Willebrand's disease with a VHP vWF concentrate: the French experience. Haemophilia 1998;4 (Suppl 3):48-52.
  394. Bunnell BA, Muul LM, Donahue RE, Blaese RM, Morgan RA. High-efficiency retroviral-mediated gene transfer into human and nonhuman primate peripheral blood lymphocytes. Proc Natl Acad Sci U S A 1995 Aug;92(17):7739-7743.
  395. Blaese RM. Optimism regarding the use of RNA/DNA hybrids to repair genes at high efficiency. J Gene Med 1999 Mar;1(2):144-147.
  396. Taubes G. Gene therapy. The strange case of chimeraplasty. Science 2002 Dec;298(5601):2116-2120.
  397. Zhang Z, Eriksson M, Falk G, Graff C, Presnell SC, Read MS, Nichols TC, Blombäck M, Anvret M. Failure to achieve gene conversion with chimeric circular oligonucleotides: potentially misleading PCR artifacts observed. Antisense Nucleic Acid Drug Dev 1998 Dec;8(6):531-536.
  398. Todd RF 3rd, Gitlin SD, Burns LJ, Committee on Training Programs. Subspeciality training in hematology and oncology, 2003: results of a survey of training program directors conducted by the American Society of Hematology. Blood 2004 Jun;103(12):4383-4388.

Back to Top
Back to Table of Contents

Twitter iconTwitterimage of external icon Facebook iconFacebookimage of external icon YouTube iconYouTubeimage of external icon Google+ iconGoogle+image of external icon