STUDIES OF THE PATHO-PHYSIOLOGY OF ACQUIRED VONWILLEBRANDS DISEASE IN 7 PATIENTS WITH LYMPHOPROLIFERATIVE DISORDERS OR BENIGN MONOCLONAL GAMMOPATHIES

  • 1 January 1984
    • journal article
    • research article
    • Vol. 64  (3) , 614-621
Abstract
In 7 patients with acquired von Willebrand''s disease (AvWD) associated with lymphoproliferative disorders or benign monoclonal gammopathies, the platelet contents of von Willebrand factor antigen and ristocetin cofactor (vWF:Ag and vWF:RiCof, respectively) were normal. All the multimers of vWF:Ag could be seen in the 1.6% SDS[sodium dodecyl sulfate]-agarose gel electrophoresis patterns of plasma and platelet lysates. Infusion of 1-deamino-8-D-arginine vasopressin (DDAVP) augmented plasma levels of vWF:Ag and vWF:RiCof of all patients and corrected prolonged bleeding times (BT). Compared with patients with congenital vWD type I and comparable degrees of baseline abnormalities treated in the same way, vWF:Ag and vWF-RiCof were increased less and cleared more rapidly from plasma and the BT remained normal for a shorter period of time. These AvWD patients apparently have qualitatively normal vWF in plasma, but at lower concentrations, that vWF in platelets in normal both qualitatively and quantitatively, and cellular vWF can be rapidly released into plasma by DDAVP to correct the hemostatic abnormalities. vWF is removed rapidly from plasma, making the correction more transient than in congenital vWD type 1.

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