Thromb Haemost 1998; 80(03): 495-498
DOI: 10.1055/s-0037-1615235
Rapid Communications
Schattauer GmbH

Quantitative Analysis of von Willebrand Factor and Its Propeptide in Plasma in Acquired von Willebrand Syndrome

Perry J. J. van Genderen
1   From the Department of Hematology, University Hospital Dijkzigt, Rotterdam, Central Laboratory of the Netherlands Red Cross Blood Transfusion Service, Amsterdam, The Netherlands
,
Ria C. Boertjes
1   From the Department of Hematology, University Hospital Dijkzigt, Rotterdam, Central Laboratory of the Netherlands Red Cross Blood Transfusion Service, Amsterdam, The Netherlands
,
Jan A. van Mourik
1   From the Department of Hematology, University Hospital Dijkzigt, Rotterdam, Central Laboratory of the Netherlands Red Cross Blood Transfusion Service, Amsterdam, The Netherlands
› Author Affiliations
Supported in part by the Dutch Thrombosis Foundation
Further Information

Publication History

Received 17 September 1997

Accepted after resubmission 07 May 1998

Publication Date:
08 December 2017 (online)

Summary

Measurement of the von Willebrand factor (vWF) propeptide, also known as von Willebrand antigen II, has been suggested to be helpful in the discrimination of congenital von Willebrand disease type I from type 2 and in assessing the extent of activation of the endothelium. We performed a quantitative analysis of mature vWF and its propeptide in plasma in 8 patients with acquired von Willebrand syndrome (AvWS) and in 20 normal individuals. Mature vWF levels were significantly lower in AvWS as compared with normal individuals (13.4 ± 3.5 vs 35.6 ± 3.3 nM, p <0.001). In contrast, propeptide levels were significantly higher in AvWS (11.4 ± 1.1 vs 4.7 ± 0.2 nM, p < 0.001), probably reflecting a compensatory increase in vWF synthesis or increased perturbation of the endothelium in AvWS. After treatment with DDAVP, propeptide and mature vWF levels rose 5-fold in AvWS, whereas propeptide levels were not altered by the infusion of a vWF concentrate or treatment with high dose intravenous immunoglobulins, indicating that plasma propeptide levels are a reliable reflection of vWF synthesis. Measurement of propeptide levels may provide additional information in AvWS as to whether decreased levels of mature vWF in the circulation are due to a decrease in synthesis or due to an accelerated removal of vWF from the circulation.

 
  • References

  • 1 Fay PJ, Kawai Y, Wagner DD, Ginsburg D, Bonthron D, Ohlsson-Wilhelm BM, Chavin SI, Abraham GN, Handin RI, Orkin SH, Montgomery RR, Marder VJ. Propolypeptide of von Willebrand factor circulates in blood and is identical to von Willebrand antigen II. Science 1986; 232: 995.
  • 2 Wagner DD, Fay PJ, Sporn LA, Sinha S, Lawrence SO, Marder VJ. Divergent fates of von Willebrand factor and its propolypeptide (von Willebrand antigen II) after secretion from endothelial cells. Proc Natl Acad USA 1987; 84: 1955-9.
  • 3 Borchiellini A, Fijnvandraat K, ten Cate JW, Pajkrt D, van Deventer SJH, Pasterkamp G, Meijer-Huizinga F, Zwart-Huinink L, Voorberg J, van Mourik JA. Quantitative analysis of von Willebrand factor propeptide release in vivo: effect of experimental endotoxemia and administration of 1-deamino-8-d-arginine vasopressin in humans. Blood 1996; 88: 2951-8.
  • 4 McCarroll DR, Ruggeri ZM, Montgomery RR. Correlation between circulating levels of von Willebrand’s antigen II and von Willebrand factor: discrimination between type I and type II von Willebrand’s disease. J Lab Clin Med 1984; 103: 704-11.
  • 5 van Genderen PJJ, Vink T, Michiels JJ, van ’t Veer MB, Sixma JJ, van Vliet HHDM. Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 1994; 84: 3378-84.
  • 6 van Genderen PJJ, Michiels JJ, van der Poel, van de Luytgaarde SCPAM, van Vliet HHDM. Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia: relationship with platelet count. Ann Hematol 1994; 69: 81-4.
  • 7 Budde U, van Genderen PJJ. Acquired von Willebrand disease in patients with high platelet counts. Sem Thromb Hemost 1997; 23: 425-31.
  • 8 Hakkert BC, Rentenaar JM, van Mourik JA. Monocytes enhance endothelial von Willebrand factor release and prostacyclin production with different kinetics and dependency on intercellular contact between these two cell types. Br J Haematol 1992; 80: 495.
  • 9 Stel HV, Sakariassen KS, Scholte BJ, Veerman ECI, van der Kwast TH, de Groot PG, Sixma JJ, van Mourik JA. Characterization of 25 monoclonal antibodies to factor VIII-von Willebrand factor: relationship between ristocetin-induced platelet aggregation adn platelet adherence to subendothelium. Blood 1984; 63: 1408.
  • 10 Fay PJ. Factor VIII structure and function. Thromb Haemost 1993; 70: 63.
  • 11 Scott JP, Vokac EA, Schroeder T. et al. The von Willebrand factor propolypeptide, von Willebrand factor antigen II, distinguishes von Wille-brand syndrome (AvWS) due to the decreased synthesis of vWF from AvWS due to increased clearance of vWF [abstract]. Blood 1995; 86: 196a.
  • 12 McCarroll DR, Ruggeri ZM, Montgomery RR. The effect of DDAVP on plasma levels of von Willebrand antigen II in normal individuals and patients with von Willebrand’s disease. Blood 1984; 63: 532-5.
  • 13 van Genderen PJJ, Leenknegt H, Budde U, Michiels JJ. Acquired von Willebrand disease in myeloproliferative disorders. Leuk Lymphoma 1996; 22 (Suppl. 01) 79-82.
  • 14 van Genderen PJJ, Papatsonis DNM, Michiels JJ, Wielenga JJ, Stibbe J, Huikeshoven FJM. High-dose intravenous gammaglobulin therapy for acquired von Willebrand disease. Postgrad Med J 1994; 70: 916-20.
  • 15 van Genderen PJJ, Terpstra W, Michiels JJ, Kapteijn L, van Vliet HHDM. High-dose intravenous immunoglobulin delays clearance of von Wille-brand factor in acquired von Willebrand disease. Thromb Haemost 1995; 73: 890-1.