Absence of a bleeding tendency in severe acquired von Willebrand's disease. The role of platelet von Willebrand factor in maintaining normal hemostasis

American Journal of Clinical Pathology
J DrouinA R Giles

Abstract

A 67-year-old male with a prolonged activated partial thromboplastin time (APTT) of 43 seconds (normal, 25-40 seconds) was found to have laboratory features of von Willebrand's disease and IgA myeloma but had a normal bleeding time and no bleeding tendency. Plasma Factor VIII coagulant activity (F.VIII:C) was 80 U/L (0.08 U/mL), Factor VIII antigen (F.VIII:Ag) 70 U/L (0.07 U/mL), and von Willebrand's factor antigen (vWF:Ag) 50 U/L (0.05 U/mL) and ristocetin cofactor (vWF:RiCoF) 10 U/L (0.10 U/mL). The platelet vWF:Ag level was normal, whereas both platelet lysate and plasma vWF high molecular weight multimers were decreased. Patient plasma had no inhibitory effect on either F.VIII:C or vWF:RiCoF. However, when patient plasma was incubated with normal plasma, crossed immunoelectrophoresis for vWF:Ag demonstrated the presence of immune complexes. Infusion of 1-desamino-8-D-arginine vasopressin led to a transient correction of the plasma vWF:Ag multimer pattern. The survival of all components of vWF/F.VIII was decreased, as also occurred after cryoprecipitate. The levels of plasma F.VIII/vWF increased as the IgA values decreased after chemotherapy, whereas the platelet high molecular weight multimers remained decreased. The data s...Continue Reading

Citations

Jun 25, 1998·Irish Journal of Medical Science·B J HennessyO P Smith
Apr 16, 2003·Haemophilia : the Official Journal of the World Federation of Hemophilia·C SiakaJ Goudemand
Aug 15, 2012·Acta Haematologica·Gui-Ping LuoYu-Zhang Wu
Dec 24, 2010·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Ralf KargerVolker Kretschmer
Oct 1, 2011·International Journal of Hematology·Toshio ShigekiyoOsamu Fujino
Apr 1, 1996·Medical and Pediatric Oncology·A B Jonge Poerink-StockschladerK Hählen

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