Acquired von willebrand disease in patients with polycythemia rubra vera

Authors

  • Hiroshi Mohri MD

    Corresponding author
    1. Department of Laboratory Medicine, School of Medicine, Yokohama City University, Yokohama, Japan
    Current affiliation:
    1. c/o T.S. Zimmerman, BCR 8, Scripps Clinic and Research Foundation, 10666 North Torrey Pines Road, La Jolla, CA 92037
    • Department of Laboratory Medicine, School of Medicine, Yokohama City University, Yokohama, Japan
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Abstract

Platelet function and factor VIII complex were evaluated in ten patients with polycythemia rubra vera. Seven patients showed abnormal epinephrine-induced aggregation. The intracellular concentrations of adenosine diphosphate (ADP) were below normal, and the ratio of adenosine triphosphate (ATP)/ADP was greater than normal. In four of eight cases, there was a decrease in ristocetin cofactor activity and a reduction in the slowly migrating forms of vWF:Ag on crossed immunoelectrophoresis. Defect of large multimers of vWF:Ag was also observed. The ratio of vWF:Ag to ristocetin cofactor was elevated in these patients. Plasma from the patients had no effect on normal plasma except in one case, in which isolated IgG appeared to cause inactivation of ristocetin cofactor. Treatment with 1-deamino-8-arginine vasopressin caused correction of the vWF abnormalities with rapid return of ristocetin cofactor to baseline in some patients. The present study shows that the alterations of multimeric structure of vWF occur in more than 50% of patients with polycythemia rubra vera and are in some part due to the inhibitor specific for vWF.

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