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.
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Authors | M L Scrobohaci, M T Daniel, Y Levy, J P Marolleau, J C Brouet |
Journal | British journal of haematology
(Br J Haematol)
Vol. 84
Issue 3
Pg. 471-5
(Jul 1993)
ISSN: 0007-1048 [Print] England |
PMID | 8217799
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Antibodies, Monoclonal
- Immunoglobulin G
- Platelet Membrane Glycoproteins
- Receptors, Cell Surface
- von Willebrand factor receptor
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Topics |
- 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, metabolism)
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