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Hamostaseologie 2003; 23(03): 121-124
DOI: 10.1055/s-0037-1619589
Original Article
Schattauer GmbH

Acquired von Willebrand syndrome in myeloproliferative disorder

Case 6Akquiriertes von-Willebrand-Syndrom im Rahmen einer myeloproliferativen Erkrankung
P. Baud
1   Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
,
A. Tobler
1   Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
,
B. Lämmle
1   Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
,
L. Alberio
1   Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland
› Author Affiliations
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Publication History

Publication Date:
23 December 2017 (online)

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Summary

We present a woman (age: 57 years) with an excessive bleeding episode under acetylsalicylic acid after bone marrow puncture due to an acquired von Willebrand syndrome (avWS) in the context of a myeloproliferative disorder. The laboratory features showed a high platelet concentration and a qualitative defect of von Willebrand factor (vWF) with a low normal vWF ristocetin cofactor activity, a normal vWF antigen and a decrease of the larger vWF multimers in plasma.

The exact mechanism of avWS is still incompletely resolved. Myeloproliferative diseases are one of several underlying disorders that may cause avWS. The diagnosis of the underlying disease is important because its treatment may lead to an improvement of the vWF abnormality. For symptomatic treatment of bleeding, desmopressin, vWF concentrate infusion, intravenous immunoglobulin and/or fibrinolysis inhibitors can be tried.

Zusammenfassung

Der Fall einer 57-jährigen Frau mit einer Blutungsepisode nach Knochenmarkpunktion unter Azetylsalizylsäure wird vorgestellt. Die Laboruntersuchungen zeigten eine Thrombozytose und einen qualitativen Defekt des von-Willebrand-Faktors (vWF) mit einer tiefnormalen vWF-Ristocetin-Kofaktoraktivität, einem normalen vWF-Antigen und einer Verminderung der hochmolekularen vWF-Multimere, entsprechend einem akquirierten von-Willebrand-Syndrom (avWS) im Rahmen einer myeloproliferativen Krankheit.

Der exakte Mechanismus des avWS ist nicht vollständig geklärt. Myeloproliferative Erkrankungen gehören zu den möglichen Ursachen. Die Identifizierung der Grundkrankheit ist wichtig, weil deren erfolgreiche Behandlung das avWS beheben kann. Für eine symptomatische avWS-Behandlung stehen Desmopressin, vWF-haltige Faktor-VIII-Konzentrate, intravenös zu verabreichende Immunglobuline und Fibrinolysehemmer zur Verfügung.

Keywords

Myeloproliferative disorder - von Willebrand factor - acquired von Willebrand syndrome - bleeding disorder

Schlüsselwörter

Myeloproliferative Erkrankungen - von-Willebrand-Faktor - akquiriertes von-Willebrand-Syndrom - hämorrhagische Diathese - Blutungsneigung
 

  • References

  • 1 Alberio L. Von Willebrand disease: a review. Eur J Int Med 1998; 9: 225-38.
    PubMedSearch in Google Scholar
  • 2 Baerlocher GM, Leoncini-Franscini L, Wuillemin WA. et al. Rezidivierende gastrointestinale Blutungen bei akquiriertem Morbus von Willebrand. Ther Umsch 1999; 56: 487-90.
    CrossrefPubMedSearch in Google Scholar
  • 3 Bracey AW, Wu AH, Aceves J. et al. Platelet dysfunction associated with Wilms tumor and hyaluronic acid. Am J Hematol 1987; 24: 247-57.
    CrossrefPubMedSearch in Google Scholar
  • 4 Bruggers CS, McElligott K, Rallison ML. Acquired von Willebrand disease in twins with autoimmune hypothyroidism: response to desmopressin and L-thyroxine therapy. J Pediatr 1994; 125: 911-3.
    CrossrefPubMedSearch in Google Scholar
  • 5 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: 227-38.
    Thieme ConnectPubMedSearch in Google Scholar
  • 6 Budde U, Scharf RE, Franke P. et al. Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood 1993; 82: 1749-57.
    PubMedSearch in Google Scholar
  • 7 Budde U, van Genderen PJ. Acquired von Willebrand disease in patients with high platelet counts. Semin Thromb Hemost 1997; 23: 425-31.
    Thieme ConnectPubMedSearch in Google Scholar
  • 8 de Romeuf C, Mazurier C. Comparison between von Willebrand factor (vWF) and vWF antigen II in normal individuals and patients with von Willebrand disease. Thromb Haemost 1998; 80: 37-41.
    Thieme ConnectPubMedSearch in Google Scholar
  • 9 Eikenboom JC, van der Meer FJ, Briet E. Acquired von Willebrand’s disease due to excessive fibrinolysis. Br J Haematol 1992; 81: 618-20.
    CrossrefPubMedSearch in Google Scholar
  • 10 Federici AB, Stabile F, Castaman G. et al. 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.
    CrossrefPubMedSearch in Google Scholar
  • 11 Gill JC, Wilson AD, Endres-Brooks J. et al. Loss of the largest von Willebrand factor multimers from the plasma of patients with congenital cardiac defects. Blood 1986; 67: 758-61.
    PubMedSearch in Google Scholar
  • 12 Goudemand J, Samor B, Caron C. et al. Acquired type II von Willebrand’s disease: demonstration of a complexed inhibitor of the von Willebrand factor-platelet interaction and response to treatment. Br J Haematol 1988; 68: 227-33.
    CrossrefPubMedSearch in Google Scholar
  • 13 Messinezy M, Pearson TC. The classification and diagnostic criteria of the erythrocytoses (polycythaemias). Clin Lab Haematol 1999; 21: 309-16.
    CrossrefPubMedSearch in Google Scholar
  • 14 Michiels JJ, Budde U, van der Planken M. et al. Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol 2001; 14: 401-36.
    CrossrefPubMedSearch in Google Scholar
  • 15 Nichols WC, Ginsburg D. von Willebrand disease. Medicine (Baltimore) 1997; 76: 1-20.
    CrossrefPubMedSearch in Google Scholar
  • 16 O’Brien JR, Etherington MD. Heart valve stenosis and von Willebrand’s factor multimers. Lancet 1992; 340: 616.
    PubMedSearch in Google Scholar
  • 17 Pareti FI, 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: 1290-5.
    CrossrefPubMedSearch in Google Scholar
  • 18 Richard C, Cuadrado MA, Prieto M. et al. Acquired von Willebrand disease in multiple myeloma secondary to absorption of von Willebrand factor by plasma cells. Am J Hematol 1990; 35: 114-7.
    CrossrefPubMedSearch in Google Scholar
  • 19 Scrobohaci ML, Daniel MT, Levy Y. et al. Expression of GpIb on plasma cells in a patient with monoclonal IgG and acquired von Willebrand disease. Br J Haematol 1993; 84: 471-5.
    CrossrefPubMedSearch in Google Scholar
  • 20 Uehlinger J, Rose E, Aledort LM. et al. Successful treatment of an acquired von Willebrand factor antibody by extracorporeal immunoadsorption. N Engl J Med 1989; 320: 254-5.
    CrossrefPubMedSearch in Google Scholar
  • 21 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: 495-8.
    Thieme ConnectPubMedSearch in Google Scholar
  • 22 van Genderen PJ, Prins FJ, Lucas IS. et al. Decreased half-life time of plasma von Willebrand factor collagen binding activity in essential thrombocythaemia: normalization after cytoreduction of the increased platelet count. Br J Haematol 1997; 99: 832-6.
    CrossrefPubMedSearch in Google Scholar
  • 23 van Genderen PJ, van Vliet HH, Prins FJ. et al. Excessive prolongation of the bleeding time by aspirin in essential thrombocythemia is related to a decrease of large von Willebrand factor multimers in plasma. Ann Hematol 1997; 75: 215-20.
    CrossrefPubMedSearch in Google Scholar
  • 24 van Genderen PJ, Vink T, Michiels JJ. et al. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 1994; 84: 3378-84.
    PubMedSearch in Google Scholar