Acquired von Willebrand's syndrome with lupus-like serology

The American Journal of Pediatric Hematology/oncology
N IgarashiN Taniguchi

Abstract

We describe a 12-year-old boy with acquired von Willebrand's syndrome, who also had various autoantibodies. He presented with recent hemorrhagic symptoms and a prolonged bleeding time. Hemostatic studies revealed severely reduced levels of factor VIII procoagulant activity (VIII:C), von Willebrand's factor (vWF) antigen (vWF:Ag), and ristocetin cofactor activity (RCoF). An inhibitor that could be detected in the patient's plasma moderately decreased the levels of vWF:Ag in normal plasma, but did not interfere with the measurement of VIII:C or RCoF. Following the infusion of cryoprecipitate, half-lives of VIII:C, vWF:Ag, and RCoF were markedly reduced. 1-Deamino-8-D-arginine vasopressin infusion induced normalization of the prolonged bleeding time and caused a marked increase in VIII:C, vWF:Ag, and RCoF. Prior to treatment, there was a uniform reduction of all the multimers of plasma vWF in sodium dodecyl sulfate agarose gel electrophoresis. Following prednisone therapy, clinical and hemostatic findings were improved, and the multimeric patterns of vWF were normalized. These findings suggest that the low levels of all three parameters of factor VIII and all the multimers of plasma vWF in the patient are caused by rapid eliminati...Continue Reading

Citations

May 29, 2002·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·M NiiyaM Tanimoto
Jul 24, 2013·Blood·Michael U CallaghanAugusto B Federici
Dec 30, 2014·Case Reports in Hematology·Sara Taveras AlamLouis J Reed
Apr 9, 2001·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·J J MichielsZ Berneman
Jun 25, 1998·Irish Journal of Medical Science·B J HennessyO P Smith
May 1, 1999·Haemophilia : the Official Journal of the World Federation of Hemophilia·P Petrini

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