Acquired von Willebrand Syndrome: Experience from 2 Years in a Single Laboratory Compared with Data from the Literature and an International Registry

Ulrich Budde; Frauke Bergmann; Jan J. Michiels

doi:10.1055/s-2002-27824

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2002; 28(2): 227-238
DOI: 10.1055/s-2002-27824

Acquired von Willebrand Syndrome: Experience from 2 Years in a Single Laboratory Compared with Data from the Literature and an International Registry

Ulrich Budde¹ , Frauke Bergmann¹ , Jan J. Michiels²

¹Coagulation Laboratory, Lab. Association Prof. Arndt and Partners, Hamburg, Germany
²Hemostasis and Thrombosis Research, Department of Hematology, University Hospital Antwerp and University Hospital Rotterdam and Goodheart Institute, Hemostasis and Thrombosis Science Center, Rotterdam, The Netherlands

Abstract

ABSTRACT

Acquired von Willebrand syndrome (avWS) has gained more attention during the last years. An International Registry has been compared with the literature. It could be shown that the data collected from 123 publications compared well with the data from the registry, albeit with differences in the number of patients suffering from lymphoproliferative diseases and cardiovascular disorders, that were more prominent in the registry and the group of miscellaneous conditions underrepresented in the registry. Our data are clearly different for the lymphoproliferative diseases with only four patients in 2 years. These patients usually suffer from severe bleeding complications together with low to very low factor VIII/von Willebrand factor (FVIII/vWF) concentrations and thus will not go undiagnosed. In contrast to this, patients with cardiovascular disorders usually bleed only during surgical procedures or catheter procedures. At that time they have increased vWF parameters. Because of this and because the acute bleeding is of limited duration and usually not life threatening, many of them are discharged without a proper diagnosis and are only rarely referred to a specialized diagnostic work-up thereafter. In conclusion, avWS, although not a frequent disease, is nevertheless clearly underdiagnosed. This should be addressed in future prospective studies.

KEYWORDS

Acquired von Willebrand syndrome - von Willebrand factor - lymphoproliferative disorders - myeloproliferative disorders - cardiovascular defects - miscellaneous conditions

Full Text

References

REFERENCES

1 Sadler J E, Mannucci P M, Berntorp E. Impact, diagnosis and treatment of von Willebrand disease. Thromb Haemost . 2000; 84 160-174
2 Ruggeri Z M. Structure and function of von Willebrand factor. Thromb Haemost . 1999; 82 576-584
3 von Willebrand A E. Hereditär pseudohemofili. Finska Läk Sällsk Handl . 1926; 68 87-112
4 Simone J V, Cornet J A, Abildgaard C F. Acquired von Willebrand's syndrome in systemic lupus erythematosus. Blood . 1968; 31 806-811
5 Ingram G IC, Kingston P J, Leslie J, Bowie E JW. Four cases of acquired von Willebrand's syndrome. Br J Haematol . 1971; 21 189-199
6 Mant M J, Gauldie H J, Bienestock G J, Pineo G F, Luke K H. Von Willebrand's syndrome presenting as an acquired bleeding disorder in association with a monoclonal gammopathy. Blood . 1973; 42 429-436
7 Joist J H, Cowan J F, Zimmerman T S. Acquired von Willebrand's disease. Evidence for a quantitative and qualitative factor VIII disorder. N Engl J Med . 1978; 298 988-991
8 Brody J I, Haidar M E, Rossman R. A hemorrhagic syndrome in Waldenström's macroglobulinemia secondary to immunoadsorption of factor VIII. N Engl J Med . 1979; 300 408-410
9 Noronha P A, Hruby M A, Maurer H S. Acquired von Willebrand disease in a patient with Wilms tumor. J Pediatr . 1979; 95 997-999
10 Budde U, Schäfer G, Müller N. Acquired von Willebrand's disease in the myeloproliferative syndrome. Blood . 1984; 64 981-985
11 Gill J C, Wilson A D, Endres-Brooks J, Montgomery R R. Loss of the largest von Willebrand factor multimers from plasma of patients with congenital cardiac defects. Blood . 1986; 67 758-761
12 Dalton R G, Dewar M S, Savidge G F. Hypothyroidism as a cause of acquired von Willebrand's disease. Lancet . 1987; 1 1007-1009
13 Kinoshita S, Yoshioka K, Kasahara M, Takamiya O. Acquired von Willebrand disease after Epstein-Barr virus infection. J Pediatr . 1991; 119 595-598
14 Jakway J L. Acquired von Willebrand's disease in malignancy. Semin Thromb Hemost . 1992; 18 434-439
15 Rinder M R, Richard R E, Rinder H M. Acquired von Willebrand's disease: a concise review. Am J Hematol . 1997; 54 139-145
16 Tefferi A, Nichols W L. Acquired von Willebrand disease: concise review of occurrence, diagnosis, pathogenesis and treatment. Am J Med . 1997; 103 536-540
17 Michiels J J, Budde U, van Genderen J J P. Acquired von Willebrand syndromes: clinical features, etiology, pathophysiology, classification and management. Baillères Clin Haematol (in press) . 2001;
18 Veyradier A, Jenkins C SP, Fressinaud E, Meyer D. Acquired von Willebrand syndrome: from pathophysiology to management. Thromb Haemost . 2000; 84 175-182
19 Federici A B, Rand J H, Bucciarelli P. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost . 2000; 84 345-349
20 Hoylaerts M F, Thys C, Arnout J, Vermylen J. Recurrent arterial thrombosis linked to autoimmune antibodies enhancing von Willebrand factor binding to platelets and inducing Fc gamma RII receptor-mediated platelet activation. Blood . 1998; 91 2810-2817
21 van Genderen J J P, Vink T, Michiels J J. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood . 1994; 84 3378-3384
22 Stewart M W, Etches W S, Shaw A RE, Gordon P A. vWF inhibitor detection by competitive ELISA. J Immunol Methods . 1997; 200 113-119
23 Zettervall O, Nilsson I M. Acquired von Willebrand's disease caused by monoclonal antibody. Acta Med Scand . 1978; 204 521-528
24 Hartleib J, Köhler N, Dickinson R B. Protein A is the von Willebrand factor binding protein on Staphylococcus aureus Blood . 2000; 96 2149-2156
25 Luboshitz J, Lubetsky A, Schliamser L. Pharmacokinetic studies with FVIII/von Willebrand factor concentrate can be a diagnostic tool to distinguish between subgroups of patients with acquired von Willebrand syndrome. Thromb Haemost . 2001; 85 806-809
26 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 2707-2711
27 Horellou M H, Baumelou E, Sitbon N. Quatre cas de deficit acquis en facteur Willebrand associés a une dysgulobulinémie monoclonale. Ann Med Interne (Paris) . 1983; 143 707-712
28 Van Genderen J J P, Terpstra W, Michiels J J, Kapteijn L, van Vliet H D M H. High-dose intravenous immunoglobulin delays clearance of von Willebrand factor in acquired von Willebrand disease. Thromb Haemost . 1995; 73 891-892
29 Gunz F W. Hemorrhagic thrombocythemia. A critical review. Blood . 1960; 15 706-723
30 Van Genderen J J P, Michiels J J. Erythromelalgic, thrombotic and hemorrhagic thrombocythemia. Press Med . 1994; 23 73-77
31 Fabris F, Casonato A, Del Ben G M, De Marco L, Girolami A. Abnormalities of von Willebrand factor in myeloproliferative disease: A relationship with bleeding diathesis. Br J Haematol . 1986; 63 75-83
32 Casonato A, Fabris F, Zancan L, Girolami A. Acquired type I von Willebrand's disease in a patient with essential thrombocytosis. Acta Haematol . 1986; 75 188-189
33 Zimmerman T S, Dent J A, Ruggeri Z M, Nannini L H. 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; 77 947-951
34 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 1213-1217
35 Van Genderen J J P, Michiels J J, van der Poel-van de Luitgaarde SC P A M, van Vliet H D M H. Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia. Ann Hematol . 1994; 69 81-84
36 Budde U, Scharf R E, Franke P. Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood . 1993; 82 749-757
37 Van Genderen J J P, Budde U, Michiels J J, van Strik R, van Vliet H D M H. The reduction of large von Willebrand factor multimers in patients with essential thrombocythemia is related to the platelet count. Br J Haematol . 1996; 93 962-965
38 Coppes M J, Zandvoort S WH, Sparling C R. Acquired von Willebrand disease in Wilms' tumor patients. J Clin Oncol . 1992; 10 422-427
39 Anderson R P, McGrath K, Street A. Reversal of aortic stenosis, bleeding gastrointestinal angiodysplasia, and von Willebrand syndrome by aortic valve replacement. Lancet . 1996; 347 689-690
40 Knobloch W, Hauser E, Niehues R. Calcifying aortic valve stenosis and cryptogenic gastrointestinal bleeding (Heyde syndrome): report of two cases. Z Kardiol . 1999; 88 448-453
41 Pareti F I, Lattuada A, Bressi C. Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis. Circulation . 2000; 102 1290-1295
42 Heyde E C. Gastrointestinal bleeding in aortic stenosis. N Engl J Med . 1958; 259 196-201
43 Warkentin T E, Moore J C, Morgan D G. Aortic stenosis and bleeding gastrointestinal angiodysplasia-is acquired von Willebrand's disease the link?. Lancet . 1992; 340 35-37
44 Van Genderen J J P, Prins F J, Lucas I S, van de Moesdijk D, van Vliet H D M H. Decreased half-life time of plasma von Willebrand factor collagen binding activity in essential thrombocythemia: normalization after cytoreduction of the increased platelet count. Br J Haematol . 1997; 99 832-836
45 Gill J C, Endres-Brooks J, Bauer P J, Marks W J, Montgomery R R. The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood . 1987; 69 1691-1695
46 Beacham D A, Lian J, Konkle B A, Ludlow L B, Shapiro S S. Arterial shear stress stimulates surface expression of the endothelial glycoprotein IB complex. J Cell Biochem . 1999; 73 508-521
47 Galbusera M, Zoja C, Donadelli R. Fluid shear stress modulates von Willebrand factor release from human vascular endothelium. Blood . 1997; 90 1558-1564
48 Rosendaal F R. High levels of factor VIII and venous thrombosis. Thromb Haemost . 2000; 83 1-2
49 Catto A J, Carter A M, Barrett J H. Von Willebrand factor and factor VIII:C in acute cerebrovascular disease-relationship to stroke subtype and mortality. Thromb Haemost . 1997; 77 1104-1108
50 Kreuz W, Linde R, Funk M. Induction of von Willebrand disease type 1 by valproic acid. Lancet . 1990; 335 178-184
51 Strauss R G, Stump D C, Henriksen R A. Hydroxyethyl starch accentuates von Willebrand's disease. Transfusion . 1985; 25 235-237
52 Benson P J, Peterson L C, Hasegawa D K, Smith C M. Abnormality of von Willebrand factor in patients with hemoglobin E-β thalassemia. Am J Clin Pathol . 1990; 93 395-399
53 Eberl W, Budde U, Bergmann F. Das erworbene von Willebrand Syndrom bei Valproattherapie ist selten. Klin Päd (Abst) . 2001; 213 S28
54 Gralnick H R, McKeown L P, Willimas S B, Shafer B C. Plasma and platelet von Willebrand factor: defects in uremia. Am J Med . 1988; 85 806-810
55 Zwaginga J J, Ijsseldijk M JW, Beeser-Visser N. High von Willebrand factor concentration compensates a relative adhesion defect in uremic blood. Blood . 1990; 75 1498-1508
56 Dentale N, Fulgaro C, Guerra L. Acquisition of factor VIII inhibitor after acute hepatitis C virus infection. Blood . 1997; 90 3233-3234