Volume 20, Issue 2 (Summer 2023)                   Sci J Iran Blood Transfus Organ 2023, 20(2): 155-177 | Back to browse issues page

XML Persian Abstract Print


Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

Rajabi A, Ahmadinejad M. Acquired von Willebrand Syndrome. Sci J Iran Blood Transfus Organ 2023; 20 (2) :155-177
URL: http://bloodjournal.ir/article-1-1478-en.html
Full-Text [PDF 889 kb]   (680 Downloads)     |   Abstract (HTML)  (1215 Views)
Full-Text:   (1371 Views)
    References:
  1. von Willebrand EA. Hereditary pseudohaemophilia. Haemophilia 1999; 5(3): 223-31.
  2. Kreuz W. von Willebrand’s disease: from discovery to therapy - milestones in the last 25 years. Haemophilia 2008, 14: 1-2.
  3. Goodeve AC. The genetic basis of von Willebrand disease. Blood Rev 2010; 24(3): 123-34.
  4. De Meyer SF, De Maeyer B, Deckmyn H, Vanhoorelbeke K. von  Willebrand  Factor:  Drug  and Drug Target. Cardiovasc Hematol Disord Drug Targets 2009; 9(1): 9-20.
  5. Leebeek FWG, Eikenboom CJ. von Willebrand’s Disease. N Engl J Med 2016; 375: 2067-80.
  6. Baronciani L, Peake I, Schneppenheim R, Goodeve A, Ahmadinejad M, Badiee Z, et al. Genotypes of European and Iranian patients with type 3 von Willebrand disease enrolled in 3WINTERS-IPS. Blood Adv 2021; 5(15): 2987-3001.
  7. Lenting PJ, Casari C, Christophe OD, Denis CV. von Willebrand factor: the old, the new and the unknown. J Thromb Haemost 2012; 10(12): 2428-37.
  8. Kubo M, Sakai K, Hayakawa M, Kashiwagi H, Yagi H, Seki Y, et al. Increased cleavage of von Willebrand factor by ADAMTS13 may contribute strongly to acquired von Willebrand syndrome development in patients with essential thrombocythemia. J Thromb Haemost 2022; 20(7): 1589-98.
  9. Lynch CJ, Cawte AD, Millar CM, Rueda D, Lane DA. A common mechanism by which type 2A von Willebrand disease mutations enhance ADAMTS13 proteolysis revealed with a von Willebrand factor A2 domain FRET construct. PLoS One 2017; 12(11): e0188405.
  10. Stockschlaeder M, Schneppenheim R, Budde U. Update on von Willebrand factor multimers: focus on high- molecular- weight  multimers  and  their  role   in hemostasis. Blood Coagul Fibrinolysis 2014; 25(3): 206-16.
  11. Levy GG, Motto DG, Ginsburg D. ADAMTS13 turns 3. Blood 2005; 106(1); 11-7.
  12. Avdonin PP, Tsvetaeva NV, Goncharov NV, Rybakova EY, Trufanov SK, Tsitrina AA, Avdonin PV. Von Willebrand Factor in Health and Disease. Biochem Moscow Suppl Ser A 2021; 15: 201-18.
  13. Wagner DD. Cell biology of von Willebrand factor. Annu Rev Cell Biol 1990; 6: 217-46.
  14. Pagliari MT, Rosendaal FR, Ahmadinejad M, Badiee Z, Baghaipour MR, Baronciani L, et al. von Willebrand factor propeptide and pathophysiological mechanisms in European and Iranian patients with type 3 von Willebrand disease enrolled in the 3WINTERS-IPS study. J Thromb Haemost 2022; 20(5): 1106-14.
  15. Seidizadeh O, Ahmadinejad M, Homayoun S, Mannucci PM, Peyvandi F. von Willebrand disease combined with coagulation defects in Iran. Blood Transfus 2021; 19(5): 428-34.
  16. Mancuso DJ, Tuley EA, Westfield LA, Worrall NK, Shelton-Inloes BB, Sorace JM, et al. Structure of the gene for human von Willebrand factor. J Biol Chem 1989; 264(33): 19514-27.
  17. Bowman  M,  Hopman  WM,   Rapson  D, Lillicrap D, James P. The prevalence of symptomatic von Willebrand disease in primary care practice. J Thromb Haemost 2010; 8(1): 213-6.
  18. Leebeek FWG, Susen S. von Willebrand disease: Clinical conundrums. Haemophilia 2018; 24(6): 37-43.
  19. Moonla C, Akkawat B, Kittikalayawong Y, Sukperm A, Meesanun M, Uaprasert N, et al. Bleeding Symptoms and von Willebrand Factor Levels: 30-Year Experience in a Tertiary Care Center. Clin Appl Thromb Hemost 2019; 25: 1-8.
  20. Morgan G, Brighton S, Laffan M, Goudemand J, Franks B, Finnegan A. The Cost of von Willebrand Disease in Europe: The CVESS Study. Clin Appl Thromb Hemost 2022; 28: 1-10.
  21. Echahdi H, El Hasbaoui B, El Khorassani M, Agadr A, Khattab M. von Willebrand's disease: case report and review of literature. Pan Afr Med J 2017; 27: 147.
  22. Roberts JC, V H Flood VH. Laboratory diagnosis of von Willebrand disease. Int J Lab Hematol 2015; 37(1): 11-7.
  23. Ng C, Motto DG, Di Paola J. Diagnostic approach to von Willebrand disease. Blood 2015; 125(13): 2029-37.
  24. James PD, Connell NT, Ameer B, Di Paola J, Eikenboom J, Giraud N, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv 2021; 5(1): 280-300.
  25. Sharma R, Flood VH. Advances in the diagnosis and treatment of von Willebrand disease. Blood 2017; 130(22): 2386-91.
  26. Favaloro EJ. Diagnosing von Willebrand Disease: A Short History of Laboratory Milestones and Innovations, Plus Current Status, Challenges, and Solutions. Semin Thromb Hemost 2014; 40: 551-70.
  27. Seidi Zadeh O, Ahmadinejad M, Amoohossein B, Homayoun S. Are Iranian patients with von Willebrand disease type 2N properly differentiated from hemophilia A and do they receive appropriate treatment? Blood Coagul Fibrinolysis 2020; 31(6): 382-6.
  28. Tiede A, Rand JH, Budde U, Ganser A, Federici AB. How I treat the acquired von Willebrand syndrome. Blood 2011; 117(25): 6777-85.
  29. Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJJ, Mohri H, et al. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000; 84(2): 345-9.
  30. Tiede A, Priesack J, Werwitzke S, Bohlmann K, Oortwijn B, Lenting P, et al. Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study. J Thromb Haemost 2008; 6(4): 569-76.
  31. Simone JV, Cornet JA, Abildgaard CF. Acquired von Willebrand’s syndrome in systemic lupus erythematosus. Blood 1968; 31(6): 806-12.
  32. Ingram GI, Kingston PJ, Leslie J, Bowie EJ. Four cases of acquired von Willebrand's syndrome. Br J Haematol 1971; 21(2): 189-99.
  33. Budde U, Bergmann F, Michiels JJ. 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(2): 227-38.
  34. Federici AB. Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders. Semin Hematol 2006; 43(1, Suppl 1): S48-S58.
  35. Kumar S, Pruthi RK, William L, Nichols WL. Acquired von Willebrand’s Syndrome: A Single Institution Experience. Am J Hematol 2003; 72(4): 243-7.
  36. Kumar S, Pruthi RK, Nichols W. Acquired von Willebrand disease. Mayo Clin Proc 2002; 77(2): 181-7.
  37. Petricevic M, Knezevic J, Samoukovic G, Bradaric B, Safradin I, Mestrovic M, et al. Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature. Thorac Cardiovasc Surg 2020; 68(3): 200-11.
  38. De Larochellière H, Puri R, Eikelboom JW, Rodés-Cabau J. Blood Disorders in Patients Undergoing Transcatheter  Aortic  Valve  Replacement: A Review. JACC Cardiovasc Interv 2019; 12(1): 1-11.
  39. Schlagenhauf A, Kalbhenn J, Geisen U, Beyersdorf F, Zieger B. Acquired von Willebrand Syndrome and Platelet Function Defects during Extracorporeal Life Support (Mechanical Circulatory Support). Hamostaseologie 2020; 40(2): 221-5.
  40. Thomas J, Kostousov V, Teruya J. Bleeding and thrombotic complications in the use of extracorporeal membrane oxygenation. Semin Thromb Hemost 2018; 44(1): 20-9.
  41. Icheva V, Ebert J, Budde U, Wiegand G, Schober S, Engel J, et al. Perioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease. Blood 2023; 141(1): 102-10.
  42. Shetty S, Kasatkar P, Ghosh K. Pathophysiology of acquired von Willebrand disease: a concise review. Eur J Haematol 2011; 87: 99-106.
  43. Cuker A, Connors JM, Katz JT, Levy BD, Loscalzo J. Clinical problem-solving. A bloody mystery. N Engl J Med 2009; 361(19): 1887-94.
  44. Leebeek FWG. New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome. Hemasphere 2021; 5(6): e586.
  45. Ichinose A, Osaki T, Souri M, Favaloro EJ. A Review of Autoimmune Acquired von Willebrand Factor Deficiency in Japan. Semin Thromb Hemost 2022; 48(8): 911-25.
  46. Kasatkar P, Ghosh K, Shetty S. Acquired von Willebrand syndrome: A rare disorder of heterogeneous etiology. J Postgrad Med 2013; 59(2): 98-101.
  47. Denis CV, Susen S, Lenting PJ. von Willebrand disease: what does the future hold? Blood 2021; 137(17): 2299-306.
  48. Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F, et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003; 349(4): 343-9.
  49. Tanaka H, Nagai Y, Kuwabara C, Shimizu R, Umeki A, Yamamoto T. Acquired von Willebrand Syndrome due to Aortic Valve Stenosis in a Case with Antiphospholipid Antibody. Intern Med 2018, 57: 1641-4.
  50. Meyer AL, Malehsa D, Bara C, Budde U, Slaughter MS, Haverich A, et al. Acquired von Willebrand syndrome in patients with an axial flow left ventricular assist device. Circ Heart Fail 2010; 3(6): 675-81.
  51. Muslem R, Caliskan K, Leebeek FWG. Acquired coagulopathy in patients with left ventricular assist devices. J Thromb Haemost 2018; 16: 429-40.
  52. Geisen U, Heilmann C, Beyersdorf F, Benk C, Berchtold-Herz M, Schlensak C, et al. Nonsurgical bleeding in patients with ventricular assist devices could be explained by acquired von Willebrand disease. Eur J Cardiothorac Surg 2008; 33(4): 679-84.
  53. Uriel N, Pak SW, Jorde UP, Jude B, Susen S, Vincentelli A, et al. Acquired von Willebrand syndrome after continuous-flow mechanical device support contributes to a high prevalence of bleeding during long-term support and at the time of transplantation. J Am Coll Cardiol 2010; 56(15): 1207-13.
  54. Horiuchi H, Doman T, Kokame K, Saiki Y, Matsumoto M. Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases. J Atheroscler Thromb 2019; 26(4): 303-14.
  55. Witt DR, McGillivray BC, Allanson JE, Hughes HE, Hathaway WE, Zipursky A, Hall JG. Bleeding diathesis in Noonan syndrome: a common association. Am J Med Genet 1988; 31(2): 305-17.
  56. Wiegand G, Hofbeck M, Zenker M, Budde U, Rauch R. Bleeding diathesis in Noonan syndrome: is acquired von Willebrand syndrome the clue? Thromb Res 2012; 130(5): e251-4.
  57. Franchini M, Mannucci PM. Acquired von Willebrand syndrome: focused for hematologists. Haematologica 2020; 105(8): 2032-7.
  58. Mohri H, Motomura S, Kanamori H, Matsuzaki M, Watanabe S, Maruta A, et al. Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood 1998; 91(10): 3623-9.
  59. Dicke C, Schneppenheim S, Holstein K, Spath B, Bokemeyer C, Dittmer R, et al. Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias. Ann Hematol 2016; 95(6): 945-57.
  60. Djunic I, Elezovic I, Ilic V, Tomin D, Suvajdzic-Vukovic N, Bila J, et al. Acquired von Willebrand syndrome in multiple myeloma. Hematology 2011; 16(4): 209-12.
  61. Shah CP, Delaune J, Mandernach MW. Acquired von Willebrand syndrome in association with multiple myeloma: remission after stem cell transplant. BMJ Case Rep 2021; 14(1): e239053.
  62. Mayne ES, Tait M, Jacobson BF, Pillay E, Louw SJ. Combination of acquired von Willebrand syndrome (AVWS) and Glanzmann thrombasthenia in monoclonal gammopathy of uncertain significance (MGUS), a case report. Thromb J 2018; 16: 30.
  63. Innocenti I, Morelli F, Autore F, Tomasso A, Corbingi A, Bellesi S, et al. Remission of acquired von Willebrand syndrome in a patient with chronic lymphocytic leukemia treated with venetoclax. Leuk Lymphoma 2019; 60(12): 3078-80.
  64. Hegerova L, He F, Zantek ND, Vercellotti GM, Holtan SG, Reding MT. Reversal of acquired von Willebrand syndrome with allogeneic stem cell transplant for chronic lymphocytic leukemia. Blood Cells Mol Dis 2019; 77: 109-12.
  65. Wolfe Z, Lash B. Acquired von Willebrand Syndrome in IgM Monoclonal Gammopathy as the Presentation of Lymphoplasmacytic Lymphoma. Case Rep Hematol 2017; 2017: 9862620.
  66. Qamar H, Lee A, Valentine K, Skeith L, Jimenez-Zepeda VH. Acquired von Willebrand Syndrome Associated to Secondary IgM MGUS Emerging after Autologous Stem Cell Transplantation for AL Amyloidosis. Mediterr J Hematol Infect Dis 2017; 9(1): e2017034.
  67. Trotti C, Sant'Antonio E, Vanni D, Casetti IC, Borsani O, Pietra D, et al. Acquired von Willebrand syndrome in myeloproliferative neoplasms with extreme thrombocytosis. Hematol Oncol 2021; 39(4): 589-92.
  68. Janjetovic S, Rolling CC, Budde U, Schneppenhem S, Schafhausen P, Peters MC, et al. Evaluation of different diagnostic tools for detection of acquired von Willebrand syndrome in patients with polycythemia vera or essential thrombocythemia. Thromb Res 2022; 218: 35-43.
  69. Rottenstreich A, Kleinstern G, Krichevsky S, Varon D, Lavie D, Kalish Y. Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera. Eur J Intern Med 2017; 41: 49-54.
  70. Knöfler R, Lange BS, Paul F, Tiebel O, Suttorp M. Bleeding signs due to acquired von Willebrand syndrome at diagnosis of chronic myeloid leukaemia in children. Br J Haematol 2020; 188(5): 701-6.
  71. Mital A, Prejzner W, Hellmann A. Acquired von Willebrand Syndrome During the Course of Myelofibrosis: Analysis of 32 Cases. Adv Clin Exp Med 2015; 24(6): 1001-6.
  72. Fukatsu M, Ohkawara H, Takahashi H, Mori H, Yaginuma M, Endo M, et al. A Case of Acquired von Willebrand Syndrome Complicated by Acute Myelomonocytic Leukemia. Case Rep Oncol 2021; 14(2): 1152-8.
  73. Stein BL, Martin K. From Budd-Chiari syndrome to acquired von Willebrand syndrome: thrombosis and bleeding complications in the myeloproliferative neoplasms. Blood 2019; 134(22): 1902-11.
  74. Stufano S, Baronciani L, Biguzzi E, Cozzi G, Colpani P, Chisini M, Peyvandi F. Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus. Haemophilia 2019; 25(1): e30-e32.
  75. Alam ST, Alexis K, Sridharan A, Strakhan M, Elrafei T, Gralla RJ, Reed LJ. Acquired Von Willebrand's Syndrome in Systemic Lupus Erythematosus. Case Rep Hematol 2014; 2014: 208597.
  76. Cao XY, Li MT, Zhang X, Zhao Y, Zeng XF, Zhang FC, et al. Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center. J Clin Rheumatol 2021; 27(5): 201-5.
  77. Hernández-Gilsoul T, Atisha-Fregoso Y, Vargas-Ruíz AG, Rivero-Sigarroa E, Dominguez-Cherit G, Namendys-Silva SA. Pulmonary hypertension secondary to hyperviscosity in a patient with rheumatoid arthritis and acquired von Willebrand disease: a case report. J Med Case Rep 2013; 7: 232.
  78. Yoshida H, Arai K, Wakashin M. Development of acquired von Willebrand's disease after mixed connective tissue disease. Am J Med 1988; 85(3): 445-6.
  79. Ott HW, Perkhofer S, Coucke PJ, de Paepe A, Spannag M. Identification of von Willebrand disease type 1 in a patient with Ehlers-Danlos syndrome classic type. Haemophilia 2016; 22(4): e309-11.
  80. Jackson SC, Odiaman L, Card RT, van der Bom JG, Poon MC. Suspected collagen disorders in the bleeding disorder clinic: a case-control study. Haemophilia 2013; 19(2): 246-50.
  81. Hanley D, Arkel YS, Lynch J, Kamiyama M. Acquired von Willebrand's syndrome in association with a lupus-like anticoagulant corrected by intravenous immunoglobulin. Am J Hematol 1994; 46(2): 141-6.
  82. Pardos-Gea J, Martínez F, Abrisqueta P, Santamaría A, Bosch F. Acquired von Willebrand syndrome in a patient with small lymphocytic lymphoma and Sjögren's syndrome: which associated condition should be prioritized? Blood Coagul Fibrinolysis 2019; 30(5): 239-42.
  83. Korsten P, Wallbach M, Binder C. Type 2A von Willebrand disease and systemic sclerosis: Vonicog alfa reduced gastrointestinal bleeding. Res Pract Thromb Haemost 2020; 4(7): 1230-4.
  84. Baxter PA, Nuchtern JG, Guillerman RP, Mahoney DH, Teruya J, Chintagumpala M, Yee DL. Acquired von Willebrand syndrome and Wilms tumor: not always benign. Pediatr Blood Cancer 2009; 52(3): 392-4.
  85. Mital A, Prejzner W, Hellmann A. Acquired von Willebrand syndrome during systemic mastocytosis: an analysis of 21 cases. Pol Arch Intern Med 2018; 128(7): 491-3.
  86. Stuijver DJF, Piantanida E, van Zaane B, Galli L, Romualdi E, Tanda ML, et al. Acquired von Willebrand syndrome in patients with overt hypothyroidism: a prospective cohort study. Haemophilia 2014; 20(3): 326-32.
  87. Flot C, Oliver I, Caron P, Savagner F, Tauber M, Claeyssens S, Edouard T. Acquired von Willebrand's syndrome caused by primary hypothyroidism in a 5-year-old girl. J Pediatr Endocrinol Metab 2019; 32(11): 1295-8.
  88. Casonato A, Pontara E, Vertolli UP, Steffan A, Durante C, De Marco L, et al. Plasma and platelet von Willebrand factor abnormalities in patients with uremia: lack of correlation with uremic bleeding. Clin Appl Thromb Hemost 2001; 7(2): 81-6.
  89. Mitrovic M, Elezovic I, Miljic P, Suvajdzic N. Acquired von Willebrand syndrome in patients with Gaucher disease. Blood Cells Mol Dis 2014; 52(4): 205-7.
  90. Prochorec-Sobieszek M, Windyga J, Maryniak RK, Misiak A, Szczepanik A. Angiodysplasia as a cause of recurrent bleeding from the small bowel in patients with von Willebrand disease. Report of 4 patients. Pol J Pathol 2004; 55(4): 173-6.
  91. Eberl W, Budde U, Bentele K, Christen HJ, Knapp R, Mey A, Schneppenheim R. Acquired von Willebrand syndrome as side effect of valproic acid therapy in children is rare. Hamostaseologie 2009; 29(2): 137-42.
  92. Jonville-Béra AP, Autret-Leca E, Gruel Y. Acquired type I von Willebrand's disease associated with highly substituted hydroxyethyl starch. N Engl J Med 2001; 345(8): 622-3.
  93. Michiels JJ, Budde U, van der Planken M, van Vliet HH, Schroyens W, Berneman Z. Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol 2001; 14(2): 401-36.
  94. Federici AB. Acquired von Willebrand syndrome: is it an extremely rare disorder or do we see only the tip of the iceberg? J Thromb Haemost 2008; 6(4): 565-8.
  95. Magyari F, Kracskó B, Bedekovics J, Bereczky Z, Illés Á, Schlammadinger Á. Differential diagnostic and treatment difficulties in a patient with acquired von Willebrand syndrome. Hematology 2021; 26(1): 301-4.
  96. Favaloro EJ, 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(3): 264-71.
  97. 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; 32(5): 514-21.
  98. Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, Mohri H, et al. On behalf of the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis: Scientific and Standardization Committee Communication. Scientific Report of the Registry on Acquired von Willebrand Syndrome: Recommendations for Diagnosis and Management. Posted on ISTH Website 2 November, 2000. Available from: http://www. med.unc. edu/isth/ ssc/ communications/ von_willebrand/vwf.htm; accessed 30 January 2008.
  99. Eikenboom J, Federici AB, Dirven RJ, Castaman G, Rodeghiero F, Budde U, et al. MCMDM-1VWD Study Group. VWF propeptide and ratios between VWF, VWF propeptide, and FVIII in the characterization of type 1 von Willebrand disease. Blood 2013; 121(12): 2336-9.
  100. Lee A, Sinclair G, Valentine K, James P, Poon MC.
    Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. Blood 2014; 124(5): e1-3.
  101. 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; 80(3): 495-8.
  102. Mannucci PM. New therapies for von Willebrand disease. Blood Adv 2019; 3(21): 3481-7.
  103. Budde U, Schneppenheim R, Eikenboom J, Goodeve A, Will K, Drewke E, et al. Detailed von Willebrand factor multimer analysis in patients with von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease (MCMDM-1VWD). J Thromb Haemost 2008; 6(5): 762-71.
  104. Michiels JJ, Berneman Z, Gadisseur A, van der Planken M, Schroyens W, Ulrich Budde U, van Vliet HHDM. Immune-mediated etiology of acquired von Willebrand syndrome in systemic lupus erythematosus and in benign monoclonal gammopathy: therapeutic implications. Semin Thromb Hemost 2006; 32(6): 577-88.
  105. Siaka C, Rugeri L, Caron C, Goudemand J. A new ELISA assay for diagnosis of acquired von Willebrand syndrome. Haemophilia 2003; 9(3): 303-8
  106. van Genderen PJ, Vink T, Michiels JJ, van’t Veer MB, Sixma JJ, van Vliet HH. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 1994; 84: 3378–84.
  107. Mohri H, Tanabe J, OhtsukaM, Yoshida M, Motomura S, Nishida S, et al. Acquired von Willebrand disease associated with multiple myeloma; characterization of an inhibitor to von Willebrand factor. Blood Coagul Fibrinolysis 1995; 6(6): 561–6.
  108. Heilmann C, Geisen U, Beyersdorf F, Nakamura L, Trummer G, Berchtold-Herz M, et al. Acquired Von Willebrand syndrome is an early-onset problem in ventricular assist device patients. Eur J Cardiothorac Surg 2011; 40(6): 1328-33.
  109. Sampson B, Anderson DR, Dugal M, Ing VW, Zayed E. Acquired type 2a von Willebrand's disease: response to immunoglobulin infusion. Haemostasis 1997; 27(6): 286-9.
  110. Stufano F, Boscarino M, Bucciarelli P, Baronciani L, Maino A, Cozzi G, Peyvandi F. Evaluation of the Utility of von Willebrand Factor Propeptide in the Differential Diagnosis of von Willebrand Disease and Acquired von Willebrand Syndrome. Semin Thromb Hemost 2019; 45(1): 36-42.















Sci J Iran Blood Transfus Organ 2023;20 (2): 155-177
Review Article
 




Acquired von Willebrand Syndrome

Rajabi A.1, Ahmadinejad M.1


1Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran


Abstract
Background and Objectives
Although von Willebrand's disease (vWD) is the most common hereditary bleeding disorder in the world, the acquired type of the disease (acquired von Willebrand’s syndrome, AvWs) which is seen in some conditions, is less common. Cardiovascular diseases, use of cardiac assistive devices, lymphoproliferative and myeloproliferative disorders are among the most common disorders associated with AvWs. Despite its low prevalence, AvWS is known as a clinically important bleeding disorder.

Materials and Methods
Considering the importance of the disease, we aimed to investigate and review the disease condition, diagnostic pattern and its clinical and laboratory findings from the disease discovery to the present time by referring to 110 related articles in PubMed, Medline, Scopus and Google Scholar databases.

Results
Clinical and laboratory findings in AvWS and vWD are often similar. So, it can be difficult to distinguish between the two conditions. However, performing screening tests prior to any clinical interventions with a high risk of bleeding, such as surgery, in any individual with an AvWs-associated disorder can greatly facilitate the diagnosis of the disease. Furthermore, a distinction clue between AvWS and vWD is the late onset of AvWS without any previous family and personal history of bleeding.

Conclusions 
According to published reports, AvWS has been considered as an uncommon disorder. But it seems that its actual prevalence is not rare and compared to what was previously thought, its prevalence is increasing at least in certain groups of patients, due to awareness of specialists especially hemaologists and cardiologists.

Key words: von Willebrand Factor, Hemorrhagic Disorders, von Willebrand Diseases






Received:  12 Dec 2022
Accepted: 26 Feb 2023



Correspondence: Ahmadinejad M., MD Pathologist. Associate professor of Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine.
P.O.Box: 14665-1157, Tehran, Iran. Tel: (+9821) 82052200; Fax: (+9821) 8860155         
E-mail:
1- Acridine Orange
1- Biological safety cabinet
1- Platelet Concentrate
2- Food and Drug Administration
3- Normal Skin Flora
4- Platelet Rich Plasma-Platelet Concentrate
5- Eosin-Methylene blue
6- Thioglycolate
1- Acridine Orange
1- Biological safety cabinet
1- Platelet Concentrate
2- Food and Drug Administration
3- Normal Skin Flora
4- Platelet Rich Plasma-Platelet Concentrate
5- Eosin-Methylene blue
6- Thioglycolate
minooam@gmail.com



 
Type of Study: Review Article | Subject: Hematology
Published: 2023/07/1

Add your comments about this article : Your username or Email:
CAPTCHA

Send email to the article author


Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

© 2025 CC BY-NC 4.0 | Scientific Journal of Iran Blood Transfus Organ

Designed & Developed by : Yektaweb