RSS-Feed abonnieren
DOI: 10.1055/s-0037-1614018
Acquired von Willebrand Syndrome: Data from an International Registry
On behalf of the Subcommittee on von Willebrand FactorPublikationsverlauf
Received
06. März 2000
Accepted after revision
27. April 2000
Publikationsdatum:
14. Dezember 2017 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00035024/200002/lookinside/thumbnails/10-1055s-0037-1614018-1.jpg)
Summary
The acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings similar to those of congenital von Willebrand disease (vWD). Despite the numerous cases reported in the literature until 1999 (n = 266), large studies on AvWS are not available. Moreover, diagnosis of AvWS has been difficult and treatment empirical. These considerations prompted us to organize an international registry. A questionnaire, devised to collect specific information on AvWS, was sent to all the members of the International Society on Thrombosis and Haemostasis (ISTH), who were invited to respond if they had diagnosed cases with the AvWS cases. 156 members answered the questionnaire and 54 of them sent information on 211 AvWS cases from 50 centers. Data were compared with those already published in the literature and 25 cases already described or not correctly diagnosed were excluded. The 186 AvWS cases that qualified for the registry were associated with lymphoproliferative (48%) and myeloproliferative disorders (15%), neoplasia (5%), immunological (2%), cardiovascular (21%) and miscellaneous disorders (9%). Ristocetin cofactor activity (vWF:RCo) or collagen binding activity (vWF:CBA) were usually low in AvWS (median values 20 U/dL, range 3-150), while factor VIII coagulant activity was sometimes normal (median 25 U/dL, range 3-191). FVIII/vWF inhibiting activities were present in only a minority of cases (16%). Bleeding episodes in AvWS were mostly of mucocutaneous type (68%) and were managed by DDAVP (32%), FVIII/vWF concentrates (37%), intravenous immunoglobulins (33%), plasmapheresis (19%), corticosteroids (19%) and immunosuppressive or chemotherapic agents (35%). Based upon the data of this international registry, it appears that AvWS is especially frequent in lympho-or myeloproliferative and cardiovascular diseases. Therefore, AvWS should be suspected and searched with the appropriate laboratory tests especially when excessive bleeding occurs in patients with these disorders. On the basis of the information provided by this registry guidelines for diagnosis and management of the AvWS are given.
-
References
- 1 Simone JV, Cornet JA, Abildgaard CF. Acquired von Willebrand’s syndrome in systemic lupus erythematosus. Blood 1968; 31: 806-12.
- 2 Mannucci PM, Mari D. Antibodies to factor VIII/von Willebrand factor in congenital and acquired von Willebrand’s disease. Prog Clin Biol Res 1984; 150: 109-22.
- 3 Glaspy JA. Hemostatic abnormalities in multiple myeloma and related disorders. Hemat /Oncol Clinics N Amer 1992; 06: 1301-14.
- 4 Jakway JL. Acquired von Willebrand’s disease in malignancy. Sem Thromb Hemost 1992; 18: 434-9.
- 5 Jakway JL. Acquired von Willebrand’s disease. Hematol Oncol Clin North Am 1992; 06: 1409-19.
- 6 Rinder MR, Richard RE, Rinder HM. Acquired von Willebrand’s disease: a concise review. Am J Hematol 1997; 54: 139-45.
- 7 Tefferi A, Nichols WL. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis and treatment. Am J Med 1997; 103: 536-40.
- 8 van Genderen PJJ, Michiels JJ. Acquired von Willebrand disease. Ballière’s Clinical Haematology 1998; 03: 319-30.
- 9 Nitu-Whalley IC, Lee CA. Acquired von Willebrand syndrome – report of 10 cases and review of the literature. Haemophilia 1999; 05: 318-26.
- 10 Dalton RG, Dewar MS, Savidge GF, Kernoff PBA, Matthews KB, Greaves M, Preston FE. Hypothyroidism as a cause of acquired von Willebrand’s disease. Lancet 1987; 01: 1007-9.
- 11 Mannucci PM, Lombardi R, Bader R, Horellou MH, Finazzi G, Besana C, Conard J, Samama M. Studies of the pathophysiology of acquired von Willebrand disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies. Blood 1984; 64: 614-21.
- 12 Noronha PA, Hruby MA, Maurer HS. Acquired von Willebrand disease in a patient with Wilms tumor. J Pediatr 1979; 95: 997-9.
- 13 Scott JP, Montgomery RR, Tubergen DG, Hays T. Acquired von Willebrand’s disease in association with Wilm’s tumor: regression following treatment. Blood 1981; 58: 665-9.
- 14 Richard C, Sedano MC, Cuadrado MA, Recio M, Hermosa V, Zubizarreta A. Acquired von Willebrand’s syndrome associated with hydatid disease of the spleen-disappearance after splenectomy. Thromb Haemost 1984; 52: 90-3.
- 15 Cappell MS, Lebwohl O. Cessation of recurrent bleeding from gastrointestinal angiodysplasia after aortic valve replacement. Ann Int Med 1986; 105: 54-7.
- 16 Weinstein M, Ware JA, Troll JH, Salzman EW. Changes in von Willebrand factor during cardiac surgery: effect of desmopressin acetate. Blood 1988; 71: 1648-55.
- 17 Coppes MJ, Zandvoort SWH, Sparling CR, Poon AO, Weitzman S, Blanchette VS. Acquired von Willebrand disease in Wilms’ tumor patients. J Clin Onc 1992; 10: 422-7.
- 18 Facon T, Caron C, Courtin P, Wurtz A, Deghaye M, Bauters F, Mazurier C, Goudemand J. Acquired type II von Willebrand’s disease associated with adrenal cortical carcinoma. Br J Haematol 1992; 80: 488-94.
- 19 Anderson RP, McGrath K, Street A. Reversal of aortic stenosis, bleeding gastrointestinal angiodysplasia, and von Willebrand syndrome by aortic valve replacement. Lancet 1996; 347: 689-90.
- 20 Duran-Saurez JR, Pico M, Zuazu J, Cortes M, Monasterio J, Tringiner J. Acquired von Willebrand’s disease caused by a chronic granulocytic leukemia. Br J Haematol 1981; 48: 173-5.
- 21 Tran TC, Mannucci PM, Schneider P, Federici AB, Bachmann F. Profound alterations of the multimeric structure of von Willebrand factor in a patient with malignant lymphoma. Br J Haematol 1985; 61: 307-14.
- 22 Murakawa M, Okamura T, Tsutsumi K, Kamura T, Shibuya T, Harada M, Niho Y. Acquired von Willebrand’s disease in association with essential thrombocythemia: regression following treatment. Acta Haematol 1992; 87: 83-7.
- 23 Budde U, van Genderen PJJ. Acquired von Willebrand disease in patients with high platelet counts. Sem Thromb Hemost 1997; 23: 425-31.
- 24 Joist JH, Cowan JF, Zimmerman TS. Acquired von Willebrand’s disease: evidence for a quantitative and qualitative factor VIII disorder. N Engl J Med 1978; 298: 988-91.
- 25 Dalrymphyle-Hay M, Aitchison R, Collins R, Sekhar M, Colvin B. Hydroxy-ethyl starch induced acquired von Willebrand’s disease. Clin Lab Haematol 1992; 14: 209-11.
- 26 Castaman G, Lattuada A, Mannucci PM, Rodeghiero F. Characterization of two cases of acquired transitory von Willebrand Syndrome with ciprofloxacin: evidence for heightened proteolysis of von Willebrand factor. Am J Hematol 1995; 49: 83-6.
- 27 Inbal A, Bank I, Zivelin A. Acquired von Willebrand disease in a patient with angiodysplasia resulting from immune-mediated clearance of von Willebrand factor. Br J Haemat 1997; 96: 179-82.
- 28 Federici AB, Stabile F, Castaman G, Canciani MT, Mannucci PM. Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood 1998; 92: 2707-11.
- 29 Scott JP, Vokac EA, Schroeder T. et al. The von Willebrand factor (vWF) propolypeptide, von Willebrand factor antigen II, distinguishes von Willebrand syndrome (AvWS) due to the decreased synthesis of vWF from AvWS due to increased clearance of vWF. [Abstract] Blood 1995; 86: 196a.
- 30 van Genderen PJJ, Boertjes RC, van Mourik JA. Quantitative analysis of von Willebrand factor and its propeptide in plasma in acquired von Willebrand syndrome. Thromb Haemost 1998; 80: 495-8.
- 31 Mohri H, Motomura S, Kanamori H, Matsuzaki M, Watanabe S, Maruta A, Kodama F, Okubo T. Clinical significance of inhibitors in acquired von Willebrand syndrome. Blood 1998; 91: 3623-9.