title: Expression of GpIb on plasma cells in a patient with monoclonal IgG and acquired von Willebrand disease.
abstract: 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.
authors.affiliation: Laboratory of Immunopathology, Hôpital Saint-Louis, Paris, France.
authors.forename: M L
authors.initials: ML
authors.lastname: Scrobohaci
authors.forename: M T
authors.initials: MT
authors.lastname: Daniel
authors.forename: Y
authors.initials: Y
authors.lastname: Levy
authors.forename: J P
authors.initials: JP
authors.lastname: Marolleau
authors.forename: J C
authors.initials: JC
authors.lastname: Brouet
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000032:analysis*; D001853:Bone Marrow / Q000737:chemistry; D006801:Humans; D007124:Immunoenzyme Techniques; D007074:Immunoglobulin G / Q000032:analysis*; D008297:Male; D010950:Plasma Cells / Q000737:chemistry*; D010980:Platelet Membrane Glycoproteins / Q000032:analysis*; D011956:Receptors, Cell Surface / Q000032:analysis*; D014842:von Willebrand Diseases / Q000276:immunology / Q000378:metabolism*
chemical_list: D000911:Antibodies, Monoclonal; D007074:Immunoglobulin G; D010980:Platelet Membrane Glycoproteins; D011956:Receptors, Cell Surface; C028254:von Willebrand factor receptor