Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: A 2013 update

Seminars in Thrombosis and Hemostasis

Volumn 39, Issue 2, 2013, Pages 191-201

  Federici, Augustob ^a   Budde, Ulrich ^b   Castaman, Giancarlo ^c   Rand, Jacobh ^d   Tiede, Andreas ^e  

^a UNIVERSITY OF MILAN   (Italy)

^b Medilys Laborgesellschaft mbH   (Germany)

^c SAN BORTOLO HOSPITAL   (Italy)

^d ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

^e HANNOVER MEDICAL SCHOOL   (Germany)

Author keywords

acquired von Willebrand syndrome; autoimmune conditions; cardiovascular diseases; inhibitors; lymphoproliferative diseases; thrombocythemia

Indexed keywords

ANTIFIBRINOLYTIC AGENT; BLOOD CLOTTING FACTOR 8; IMMUNOGLOBULIN; RECOMBINANT BLOOD CLOTTING FACTOR 7A; VON WILLEBRAND FACTOR;

ACQUIRED VON WILLEBRAND SYNDROME; ARTICLE; CARDIOVASCULAR DISEASE; CLINICAL FEATURE; CLINICAL TRIAL (TOPIC); CONGENITAL HEART DISEASE; GENE MUTATION; GENETIC SCREENING; LYMPHOPROLIFERATIVE DISEASE; MYELOPROLIFERATIVE NEOPLASM; PATHOGENESIS; PATHOPHYSIOLOGY; PLASMA TRANSFUSION; PLASMAPHERESIS; PRIORITY JOURNAL; REMISSION; THROMBOCYTHEMIA; TREATMENT DURATION; VON WILLEBRAND DISEASE;

EID: 84874190092     PISSN: 00946176     EISSN: 10989064     Source Type: Journal    
DOI: 10.1055/s-0033-1334867     Document Type: Article

References (72)

1. Simone J. V., Cornet J. A., Abildgaard C. F. Acquired von Willebrand's syndrome in systemic lupus erythematosus. Blood: 1968; 31 6 806 812
2. Ingram G. IC, Kingston P. J., Leslie J., Bowie E. JW. Four cases of acquired von Willebrand's syndrome. Br J Haematol: 1971; 21 2 189 199
3. Mohri H., Motomura S., Kanamori H., et al. Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood: 1998; 91 10 3623 3629 (Pubitemid 28225728)
4. Mannucci P. M., Lombardi R., Bader R., et al. Studies of the pathophysiology of acquired von Willebrand's disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies. Blood: 1984; 64 3 614 621 (Pubitemid 14073478)
5. Jakway J. L. Acquired von Willebrand's disease in malignancy. Semin Thromb Hemost: 1992; 18 4 434 439 (Pubitemid 23190622)
6. Rinder M. R., Richard R. E., Rinder H. M. Acquired von Willebrand's disease: a concise review. Am J Hematol: 1997; 54 2 139 145 (Pubitemid 27079420)
7. Federici A. B., Stabile F., Castaman G., Canciani M. T., Mannucci P. M. Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood: 1998; 92 8 2707 2711 (Pubitemid 28469082)
8. Nitu-Whalley I. C., Lee C. A. Acquired von Willebrand syndrome-report of 10 cases and review of the literature. Haemophilia: 1999; 5 5 318 326 (Pubitemid 29495708)
9. Veyradier A., Jenkins C. SP, Fressinaud E., Meyer D. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost: 2000; 84 2 175 182 (Pubitemid 30601460)
10. Federici A. B., Rand J. H., Bucciarelli P., et al. Subcommittee on von Willebrand Factor Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost: 2000; 84 2 345 349 (Pubitemid 30601485)
11. Michiels J. J., Budde U., van der Planken M., van Vliet H. H., Schroyens W., Berneman Z. Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol: 2001; 14 2 401 436 (Pubitemid 32677087)
12. Budde U., Bergmann F., Michiels J. J. Acquired von Willebrand syndrome: experience from 2 years in a single laboratory compared with data from the literature and an international registry. Semin Thromb Hemost: 2002; 28 2 227 238
13. Kumar S., Pruthi R. K., Nichols W. L. Acquired von Willebrand's syndrome: a single institution experience. Am J Hematol: 2003; 72 4 243 247 (Pubitemid 36383974)
14. Federici A. B. Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders. Semin Hematol: 2006; 43 1 01 S48 S58 (Pubitemid 43150500)
15. Tiede A., Priesack J., Werwitzke S., et al. Diagnostic workup of patients with acquired von Willebrand syndrome: a retrospective single-centre cohort study. J Thromb Haemost: 2008; 6 4 569 576 (Pubitemid 351404923)
16. Federici A. B. 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 4 565 568 (Pubitemid 351404922)
17. Tiede A., Rand J. H., Budde U., Ganser A., Federici A. B. How I treat the acquired von Willebrand syndrome. Blood: 2011; 117 25 6777 6785
18. Giannini S., Solimando M., Fierro T., Baronciani L., Federici A. B., Gresele P. Acquired von Willebrand syndrome type 2A in a JAK2-positive essential thrombocythaemia-affected member of a large von Willebrand disease family with a novel autosomal dominant A1716P mutation. Thromb Haemost: 2011; 105 5 921 924
19. van Genderen P. J., Boertjes R. C., van Mourik J. A. Quantitative analysis of von Willebrand factor and its propeptide in plasma in acquired von Willebrand syndrome. Thromb Haemost: 1998; 80 3 495 498 (Pubitemid 28438748)
20. Haberichter S. L., Castaman G., Budde U., et al. Identification of type 1 von Willebrand disease patients with reduced von Willebrand factor survival by assay of the VWF propeptide in the European study: molecular and clinical markers for the diagnosis and management of type 1 VWD (MCMDM-1VWD). Blood: 2008; 111 10 4979 4985
21. Zettervall O., Nilsson I. M. Acquired von Willebrand's disease caused by a monoclonal antibody. Acta Med Scand: 1978; 204 6 521 528 (Pubitemid 9099755)
22. Fricke W. A., Brinkhous K. M., Garris J. B., Roberts H. R. Comparison of inhibitory and binding characteristics of an antibody causing acquired von Willebrand syndrome: an assay for von Willebrand factor binding by antibody. Blood: 1985; 66 3 562 569 (Pubitemid 15230759)
23. van Genderen P. JJ, Vink T., Michiels J. J., van 't Veer M. B., Sixma J. J., van Vliet H. H. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood: 1994; 84 10 3378 3384 (Pubitemid 24352900)
24. Stewart M. W., Etches W. S., Shaw A. RE, Gordon P. A. vWf inhibitor detection by competitive ELISA. J Immunol Methods: 1997; 200 1-2 113 119 (Pubitemid 27035693)
25. Siaka C., Rugeri L., Caron C., Goudemand J. A new ELISA assay for diagnosis of acquired von Willebrand syndrome. Haemophilia: 2003; 9 3 303 308 (Pubitemid 36622033)
26. Friederich P. W., Wever P. C., Briët E., Doorenbos C. J., Levi M. Successful treatment with recombinant factor VIIa of therapy-resistant severe bleeding in a patient with acquired von Willebrand disease. Am J Hematol: 2001; 66 4 292 294 (Pubitemid 32210299)
27. Silberstein L. E., Abrahm J., Shattil S. J. The efficacy of intensive plasma exchange in acquired von Willebrand's disease. Transfusion: 1987; 27 3 234 237 (Pubitemid 17087589)
28. Tran-Thang C., Mannucci P. M., Schneider P., Federici A., Bachmann F. Profound alterations of the multimeric structure of von Willebrand factor in a patient with malignant lymphoma. Br J Haematol: 1985; 61 2 307 314 (Pubitemid 15240838)
29. Gill J. C., Wilson A. D., Endres-Brooks J., Montgomery R. R. Loss of the largest von Willebrand factor multimers from the plasma of patients with congenital cardiac defects. Blood: 1986; 67 3 758 761 (Pubitemid 16130269)
30. Heyde E. C. Gastrointestinal bleeding in aortic stenosis. N Engl J Med: 1958; 259 196
31. King R. M., Pluth J. R., Giuliani E. R. The association of unexplained bleeding with calcific aortic stenosis. Ann Thorac Surg: 1987; 44 514 516 (Pubitemid 17159755)
32. Weinstein M., Ware J. A., Troll J., Salzman E. Changes in von Willebrand factor during cardiac surgery: effect of desmopressin acetate. Blood: 1988; 71 6 1648 1655
33. Warkentin T. E., Moore J. C., Morgan D. G. Aortic stenosis and bleeding gastrointestinal angiodysplasia: is acquired von Willebrand's disease the link? Lancet: 1992; 340 8810 35 37
34. Lopes A. AB, Maeda N. Y., Aiello V. D., Ebaid M., Bydlowski S. P. Abnormal multimeric and oligomeric composition is associated with enhanced endothelial expression of von Willebrand factor in pulmonary hypertension. Chest: 1993; 104 5 1455 1460 (Pubitemid 23327863)
35. Anderson R. P., McGrath K., Street A. Reversal of aortic stenosis, bleeding gastrointestinal angiodysplasia, and von Willebrand syndrome by aortic valve replacement. Lancet: 1996; 347 9002 689 690 (Pubitemid 26076146)
36. Knobloch W., Hauser E., Niehues R., et al. Calcifying aortic valve stenosis and cryptogenic gastrointestinal bleeding (Heyde syndrome): report of two cases. Z Kardiol: 1999; 88 448 453 (Pubitemid 29315430)
37. Pareti F. I., Lattuada A., Bressi C., et al. Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis. Circulation: 2000; 102 11 1290 1295
38. Rauch R., Budde U., Girisch M., Klinge J., Hofbeck M. Acquired von Willebrand disease in an infant. Resolution by interventional occlusion of patent ductus arteriosus. Thromb Res: 2001; 102 5 407 409 (Pubitemid 32520971)
39. Veyradier A., Balian A., Wolf M., et al. Abnormal von Willebrand factor in bleeding angiodysplasias of the digestive tract. Gastroenterology: 2001; 120 2 346 353 (Pubitemid 32147631)
40. Arslan M. T., Ozyurek R., Kavakli K., et al. Frequency of acquired von Willebrand's disease in children with congenital heart disease. Acta Cardiol: 2007; 62 4 403 408 (Pubitemid 47310627)
41. Vincentelli A., Susen S., Le Tourneau T., et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med: 2003; 349 4 343 349 (Pubitemid 36886240)
42. Velik-Salchner C., Eschertzhuber S., Streif W., et al. Acquired von Willbrand syndrome in cardiac patients. J Thorac Vasc Anesth: 2007
43. Geisen U., Heilmann C., Beyersdorf F., et al. Non-surgical bleeding in patients with ventricular assist devices could be explained by acquired von Willebrand disease. Eur J Cardiothorac Surg: 2008; 33 4 679 684
44. Sánchez-Luceros A., Meschengieser S. S., Woods A. I., et al. Acquired von Willebrand factor abnormalities in myeloproliferative disorders and other hematologic diseases: a retrospective analysis by a single institution. Haematologica: 2002; 87 3 264 270 (Pubitemid 34212442)
45. Budde U., Schaefer G., Mueller N., et al. Acquired von Willebrand's disease in the myeloproliferative syndrome. Blood: 1984; 64 5 981 985 (Pubitemid 15212036)
46. Budde U., Scharf R. E., Franke P., Hartmann-Budde K., Dent J., Ruggeri Z. M. Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood: 1993; 82 6 1749 1757 (Pubitemid 23278765)
47. Van Genderen P. J., Michiels J. J. Erythromelalgic, thrombotic and hemorrhagic thrombocythemia. Presse Med: 1994; 23 73 77
48. Michiels J. J., Berneman Z., Schroyens W., Finazzi G., Budde U., van Vliet H. H. The paradox of platelet activation and impaired function: platelet-von Willebrand factor interactions, and the etiology of thrombotic and hemorrhagic manifestations in essential thrombocythemia and polycythemia vera. Semin Thromb Hemost: 2006; 32 6 589 604 (Pubitemid 44453020)
49. Fabris F., Casonato A., Grazia del Ben M., De Marco L., Girolami A. Abnormalities of von Willebrand factor in myeloproliferative disease: a relationship with bleeding diathesis. Br J Haematol: 1986; 63 1 75 83 (Pubitemid 16113732)
50. van Genderen P. JJ, Budde U., Michiels J. J., van Strik R., van Vliet H. H. The reduction of large von Willebrand factor multimers in plasma in essential thrombocythaemia is related to the platelet count. Br J Haematol: 1996; 93 4 962 965 (Pubitemid 26200180)
51. Casonato A., Fabris F., Zancan L., Girolami A. Acquired type I von Willebrand's disease in a patient with essential thrombocytosis. Acta Haematol: 1986; 75 3 188 189 (Pubitemid 16016179)
52. Budde U., Dent J. A., Berkowitz S. D., Ruggeri Z. M., Zimmerman T. S. Subunit composition of plasma von Willebrand factor in patients with the myeloproliferative syndrome. Blood: 1986; 68 6 1213 1217 (Pubitemid 17228794)
53. Shim K., Anderson P. J., Tuley E. A., Wiswall E., Sadler J. E. Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress. Blood: 2008; 111 2 651 657
54. Bangerter M., Güthner C., Beneke H., Hildebrand A., Grünewald M., Griesshammer M. Pregnancy in essential thrombocythaemia: treatment and outcome of 17 pregnancies. Eur J Haematol: 2000; 65 3 165 169
55. Ruggeri M., Rodeghiero F., Tosetto A., et al. Gruppo Italiano Malattie Ematologiche dell'Adulto (GIMEMA) Chronic Myeloproliferative Diseases Working Party Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: a retrospective survey. Blood: 2008; 111 2 666 671
56. Finazzi G., Budde U., Michiels J. J. Bleeding time and platelet function in essential thrombocythemia and other myeloproliferative syndromes. Leuk Lymphoma: 1996; 22 01 71 78 (Pubitemid 26388534)
57. Coppes M. J., Zandvoort S. WH, Sparling C. R., Poon A. O., Weitzman S., Blanchette V. S. Acquired von Willebrand disease in Wilms' tumor patients. J Clin Oncol: 1992; 10 3 422 427
58. Baxter P. A., Nuchtern J. G., Guillerman R. P., et al. Acquired von Willebrand syndrome and Wilms tumor: not always benign. Pediatr Blood Cancer: 2009; 52 3 392 394
59. Hong S., Lee J., Chi H., et al. Systemic lupus erythematosus complicated by acquired von Willebrand's syndrome. Lupus: 2008; 17 9 846 848
60. Kreuz W., Linde R., Funk M., et al. Induction of von Willebrand disease type I by valproic acid. Lancet: 1990; 335 8701 1350 1351 (Pubitemid 20186427)
61. Strauss R. G., Stump D. C., Henriksen R. A. Hydroxyethyl starch accentuates von Willebrand's disease. Transfusion: 1985; 25 3 235 237 (Pubitemid 15004522)
62. Dalton R. G., Dewar M. S., Savidge G. F., et al. Hypothyroidism as a cause of acquired von Willebrand's disease. Lancet: 1987; 1 8540 1007 1009
63. Benson P. J., Peterson L. C., Hasegawa D. K., Smith C. M. II. Abnormality of von Willebrand factor in patients with hemoglobin E-β (0) thalassemia. Am J Clin Pathol: 1990; 93 3 395 399 (Pubitemid 20103068)
64. Koenig S., Gerstner T., Keller A., Teich M., Longin E., Dempfle C. E. High incidence of vaproate-induced coagulation disorders in children receiving valproic acid: a prospective study. Blood Coagul Fibrinolysis: 2008; 19 5 375 382
65. Eberl W., Budde U., Bentele K., et al. Acquired von Willebrand syndrome as side effect of valproic acid therapy in children is rare. Hamostaseologie: 2009; 29 2 137 142
66. Franchini M., Zugni C., Veneri D., et al. High prevalence of acquired von Willebrand's syndrome in patients with thyroid diseases undergoing thyroid surgery. Haematologica: 2004; 89 11 1341 1346 (Pubitemid 39540571)
67. Gralnick H. R., McKeown L. P., Williams S. B., Shafer B. C., Pierce L. Plasma and platelet von Willebrand factor defects in uremia. Am J Med: 1988; 85 6 806 810 (Pubitemid 19020525)
68. Pasa S., Altintas A., Cil T., Danis R., Ayyildiz O., Muftuoglu E. A case of essential mixed cryoglobulinemia and associated acquired von-Willebrand disease treated with rituximab. J Thromb Thrombolysis: 2009; 27 2 220 222
69. Kos C. A., Ward J. E., Malek K., et al. Association of acquired von Willebrand syndrome with AL amyloidosis. Am J Hematol: 2007; 82 5 363 367 (Pubitemid 46651139)
70. Haj M. A., Murch N., Bowen D. J., et al. Cefotaxime as the potential cause of transient acquired von Willebrand syndrome. Eur J Haematol: 2006; 76 5 440 443
71. Mühlhausen C., Schneppenheim R., Budde U., et al. Decreased plasma concentration of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia. J Inherit Metab Dis: 2005; 28 6 945 950 (Pubitemid 43168931)
72. Sokol L., Stueben E. T., Jaikishen J. P., Lamarche M. B. Turner syndrome associated with acquired von Willebrand disease, primary biliary cirrhosis, and inflammatory bowel disease. Am J Hematol: 2002; 70 3 257 259 (Pubitemid 34680690)