Demonstration of abnormal factor VIII multimers in acquired von Willebrand's disease associated with a circulating inhibitor

Br J Haematol. 1987 Jan;65(1):95-100. doi: 10.1111/j.1365-2141.1987.tb06141.x.

Abstract

We have studied the factor VIII multimeric structure in four patients with acquired von Willebrand's disease associated with a circulating inhibitor to the factor VIII complex. In three of the four patients tested, the high and medium molecular weights bands were absent when assessed by sodium dodecyl sulphate-agarose electrophoresis. Plasma from the fourth patient contained all the factor VIII multimeric forms, although the high molecular weight bands were markedly decreased in concentration. Intravenous infusion of 1-deamino-(8-D-arginine)-vasopressin (DDAVP) resulted in the appearance of a full complement of multimers in the plasma of the two patients tested. This response was, however, transient and a return to pre-infusion multimeric composition occurred within 2-4 h. In the patients studied, an inhibitor to the factor VIII complex has induced an acquired variant von Willebrand's disease which transiently corrects after the infusion of DDAVP.

MeSH terms

  • Deamino Arginine Vasopressin / pharmacology
  • Factor VIII / analysis
  • Factor VIII / antagonists & inhibitors*
  • Genetic Variation
  • Humans
  • Immunoelectrophoresis, Two-Dimensional
  • Molecular Weight
  • von Willebrand Diseases / blood*
  • von Willebrand Diseases / genetics

Substances

  • Factor VIII
  • Deamino Arginine Vasopressin