Expression of GpIb on plasma cells in a patient with monoclonal IgG and acquired von Willebrand disease

Br J Haematol. 1993 Jul;84(3):471-5. doi: 10.1111/j.1365-2141.1993.tb03103.x.


To get insights into the pathogenesis of acquired von Willebrand disease associated with plasma cell dyscrasias, we searched for the expression of the physiological von Willebrand factor receptor, the GpIb/GpIX complex, on bone marrow plasma cells. The monoclonal spike in our patient corresponded to IgG kappa molecules; there was no plasma inhibitor to vWF:Ag or vWF:RiCoF. The bone marrow contained 1-2% plasma cells. Fresh bone marrow cells or plasma cells enriched bone marrow cells after a 48 h in vitro culture in the presence of interleukin 6 were stained by an immuno alkaline phosphatase technique using monoclonal antibodies (mAb) to von Willebrand factor, GpIb alpha and beta chain, GpIIb/IIIa and Gp IX. Two different mAb to GpIb alpha chains reacted with the majority (75%) of plasma cells whereas all other reagents yielded no staining. Malignant plasma cells from patients with multiple myeloma without haemostatic disorder were unreactive with anti-GpIb mAb. These data suggest that in some patients with acquired von Willebrand syndrome there is a GpIb mediated selective adsorption of von Willebrand factor on clonal plasma cells.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibodies, Monoclonal / analysis*
  • Bone Marrow / chemistry
  • Humans
  • Immunoenzyme Techniques
  • Immunoglobulin G / analysis*
  • Male
  • Plasma Cells / chemistry*
  • Platelet Membrane Glycoproteins / analysis*
  • Receptors, Cell Surface / analysis*
  • von Willebrand Diseases / immunology
  • von Willebrand Diseases / metabolism*


  • Antibodies, Monoclonal
  • Immunoglobulin G
  • Platelet Membrane Glycoproteins
  • Receptors, Cell Surface
  • von Willebrand factor receptor