Post-DDAVP thrombocytopenia in type 2B von Willebrand disease is not associated with platelet consumption: Failure to demonstrate glycocalicin increase or platelet activation

Thrombosis and Haemostasis

Volumn 81, Issue 2, 1999, Pages 224-228

  Casonato, A ^b,c   Steffan, A ^a   Pontara, E ^b   Zucchetto, A ^b   Rossi, C ^b   De Marco, L ^a   Girolami, A ^b  

^a CRO IRCCS Aviano   (Italy)

^b UNIVERSITY OF PADUA MEDICAL SCHOOL   (Italy)

^c UNIVERSITY HOSPITAL OF PADOVA   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

DESMOPRESSIN; GLYCOCALICIN; PADGEM PROTEIN; VON WILLEBRAND FACTOR;

ARTICLE; CLINICAL ARTICLE; HUMAN; PRIORITY JOURNAL; THROMBOCYTE ACTIVATION; THROMBOCYTE COUNT; THROMBOCYTE MEMBRANE; THROMBOCYTOPENIA; VON WILLEBRAND DISEASE;

EID: 0033065004     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1614447     Document Type: Article

References (42)

1. Ruggeri ZM, Ware J. Von Willebrand factor. Thromb Haemost 1992; 67: 594-9.
2. Sakariassen KS, Nievelstein PF, Coller BS, Sixma JJ. The role of platelet membrane glycoproteins Ib and IIb-IIIa in platelet adherence to human artery subendothelium. Nature 1987; 279: 636-8.
3. Caen JP, Nurden AT, Jeanneau C, Michel H, Tobelem G et al. Bernard-Soulier syndrome: a new platelet glycoprotein abnormality. Its relationship with platelet adhesion to subendothelium and with the factor VIII von Willebrand protein. J Lab Clin Med 1976; 87: 586-96.
4. De Marco L, Girolami A, Zimmerman TS, Ruggeri ZM. Von Willebrand factor interaction with the GPIIb-IIIa complex. Its role in platelet function as demonstrated in patients with congenital afibrinogenemia. J Clin Invest 1986; 77: 1272-7.
5. De Marco L. Mazzuccato M, De Roia D, Casonato A, Federici AB, Girolami A, Ruggeri ZM. Distinct abnormalities in the interaction of purified IIA and IIB von Willebrand factor with the two platelet binding sites, glycoprotein Ib-IX and IIb-IIIa . J Clin Invest 1990; 86: 785-92.
6. De Marco L. Mazzuccato M, Del Ben MG , Budde U, Federici AB, Girolami A, Ruggeri ZM. Interaction of purified IIB von Willebrand factor with the platelet membrane glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb-IIIa complex and initiates aggregation. Proc Nat Acad Sci 1987; 82: 7424-8.
7. Saba HI, Saba SR, Dent J, Ruggeri ZM, Zimmerman TS. Type IIB Tampa: a variant of von Willebrand disease with chronic thrombocytopenia. Circulating platelet aggregates and spontaneous platelet aggregation. Blood 1985; 66: 282-6.
8. Donner M, Holmberg L, Nilsson IM. Type IIB von Willebrand's disease with probable autosomal recessive inheritance and presenting thrombocytopenia in infancy. Brit J Haemat 1987; 66: 349-54.
9. Rick ME, Williams SB, Sacher RA, McKeown LP. Thrombocytopenia associated with pregnancy in a patient with type IIB von Willebrand's disease. Blood 1987; 3: 786-9.
10. Casonato A, Fabris F, Girolami A. Platelet aggregation and pseudothrombocytopenia induced by l-desamino-8D-arginine vasopressin (DDAVP) in type 2B von Willebrand's disease patient. Eur J Haematol 1990; 45, 36-42.
11. Weiss HJ, Sussman II. A new von Willebrand factor variant (type I, New York): increased ristocetin-induced platelet aggregation and plasma von Willebrand factor containing the full range of multimers. Blood 1986; 68: 145-56.
12. Holmberg L, Berbtorp E, Donner M, Nilsson IM. Von Willebrand's disease characterized by increased ristocetin sensitivity and the presence of all von Willebrand factor multimers in plasma. Blood 1986; 68: 668-72.
13. Goto S, Salomon DR, Ikeda Y, Ruggeri ZM. Characterization of the unique mechanism mediating the shear-dependent binding of soluble von Willebrand factor to platelets. J Biol Chem 1995; 270: 23352-61.
14. Coller BS, Kalomiris E, Steinberg M, Scudder LE. Evidence that glycocalicin circulates in normal plasma. J Clin Invest 1984; 73: 794-9.
15. Steinberg MH, John MD, Kelton JG, Barry S, Coller MD. Plasma glycocalicin: An aid in the classification of thrombocytopenic disorders. New Engl J Med 1987; 317: 1037-42.
16. Beer JH, Buchi L, Steiner B. Glycocalicin: a new assay the normal plasma levels and its potential usefulness in selected disease. Blood 1994; 83: 681-92.
17. Casonato A, De Marco L, Mazzuccato M, De Angelis V, De Roia A, Fabris F, Ruggeri ZM, Girolami A. A new congenital platelet abnormality characterized by spontaneous platelet aggregation, enhanced von Willebrand-factor platelet interaction and the presence of all von Willebrand factor multimers in plasma. Blood 1989; 74: 2028-33.
18. Girolami A, Molaro G, Lazzarin M, Scarpa R, Brunetti A. A new congenital haemorrhagic condition due to the presence of abnormal factor X (factor X Friuli): study of a large kindred. Brit J Haematol 1970; 19: 179-92.
19. Casonato A, Fabris F, Vicariotto M, Girolami A. The evaluation of factor VIII antigen by means of a simple slide test. Am J Clin Path 1985; 29: 309-16.
20. Casonato A, Sartori MT, Pontara E, Zucchetto S, Girolami A. Impaired release of tissue plasminogen activator (t-PA) following DDAVP infusion in von Willebrand's disease with low platelet von Willebrand factor content. Blood Coag Fibrin 1992; 3: 149-53.
21. Handa M, Titani K, Holland LZ, Roberts JR, Ruggeri ZM. The von Willebrand factor-binding domain of platelet membrane glycoprotein Ib. J Biol Chem 1986; 261: 12579-85.
22. Murata M, Ware J, Ruggeri ZM. Site-directed mutagenesis of a soluble recombinant fragment of platelet glycoprotein Ib-alfa demonstrating negatively charged residues involved in von Willebrand factor binding. J Biol Chem 1991; 286: 15474-80.
23. Vicente V, Kostel PJ, Ruggeri ZM. Isolation and functional characterization of the von Willebrand factor-binding domain between residues His-Arg293 of the alpha-chain of glycoprotein Ib. J Biol Chem 1988; 263: 18473-9.
24. Kunishima S, Hayashi K, Kobayashi S, Noal T, Ohno R. New enzyme-linked immunoadsorbent assay for glycocalicin in plasma. Clin Chem 1991; 37: 169-72.
25. George JN, Pickett EB, Saucerman S, McEver RP, Kunicki TJ, Kieffer N, Newman PJ. Platelet surface glycoprotein. Studies on resting and activated platelets and platelet microparticles in normal subjects, and observation in patients during adult respiratory distress syndrome and cardiac surgery. J Clin Invest 1986; 78: 340-8.
26. Mazzuccato M, De Marco L, Masotti A, Pradella P, Bahous WF, Ruggeri ZM. Characterization of the initial α-thrombin interaction with glycoprotein Ibα in relation to platelet activation. J Biol Chem 1998; 273: 1880-98.
27. Casonato A. Sartori MT, De Marco L, Girolami A. I-desamino-8-d-arginine vasopressin (DDAVP) in type IIB von Willebrand's disease: shortenening of bleeding time and induction of variable pseudothrombocytopenia. Thromb Haemost 1990; 64: 117-20.
28. De Marco L, Mazzuccato M, del Ben MG, Budde U, Federici AB, Girolami A, Ruggeri ZM. Type IIB von Willebrand factor with normal sialic acid content induces platelet aggregation in the absence of ristocetin: role of platelet activation, fibrinogen and two distinct membrane receptors. J Clin Invest 1987; 80: 475-82.
29. Gralnick HR, Williams SB, McKeown LP, Rick ME, Maisonneuve P, Jeanneau C, Sultan Y. Von Willebrand's disease with spontaneous platelet aggregation induced by an abnormal plasma von Willebrand factor. J Clin Invest 1985; 76: 1522-9.
30. Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A. DDAVP: A new pharmacological approach to the management of haemophilia A and von Willebrand disease. Lancet 1977; 1: 869-72.
31. Holmberg L, Nilsson IM, Borge L, Gunnarson M, Sjorin E. Platelet aggregation induced by l-desamino-8-d-arginine vasopressin (DDAVP) in type IIB von Willebrand's disease. N Engl J Med 1983; 309: 816-21.
32. Fowler WE, Berkowitz LR, Roberts HR. DDAVP for type IIB von Willebrand disease. Blood 1989; 74: 1859-60.
33. Connaghan DG, Gralnick HR. Beneficial response to desmopressin in type IIB von Willebrand's disease. Blood 1990; 76: 1659-59.
34. Casonato A, Pontara E, Dannhauser D, Bertomoro A, Sartori MT, Zerbinati P, Girolami A. Re-evaluation of the therapeutic efficacy of DDAVP in type IIB von Willebrand's disease. Blood Coag Fibrin 1994; 5: 959-64.
35. Castaman G, Rodeghiero F. Desmopressin and type 2B vWD. Haemophilia 1996; 2: 73-7.
36. Kyrle PA, Niessner H, Dent J, Panzer S, Brener B, Zimmerman TS, Lechner K. IIB von Willebrand's disease: pathogenetic and therapeutic studies. Brit J Haemat 1988; 69: 55-9.
37. 2+ concentration. Thromb Haemost 1993; 70: 697-701.
38. Francesconi MA, Casonato A, Pontara E, Dalla Via L, Girolami A, Deana R. Type IIB von Willebrand factor induces phospholipase A2 activation and cytosolic Ca2+ increase in platelets. Bioch Biophys Comm 1995; 24: 102-9.
39. Gralnick HR, Williams S, McKeown LP, Connaghan G, Shafer B, Hansmann K, Magruder L, Vial M. Platelet activation and alpha granule secretion in type IIB von Willebrand's disease. Brit J Haematol 1991; 79: 618-23.
40. Gangarosa EJ. Complications of ristocetin therapy. Lancet: 1958; 1: 1179.
41. Gangarosa EJ, Johnson TR, Harold SR. Ristocetin-induced thrombocytopenia: site and mechanism of action. Arch Intern Med 1960; 105: 107-13.
42. Lankhof H, Damas C, Schiphorst ME, Ijsseldijk MJW, Bracke M, Sixma JJ, Vink T, de Groot PG. Functional studies on platelet adhesion with recombinant von Willebrand factor type 2B mutants R543Q and R543W under conditions of flow. Blood 1997; 89: 2766-72.