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Misleading antigenic von Willebrand factor levels in acquired von Willebrand syndrome secondary to monoclonal gammopathy of undetermined significance. | LitMetric

AI Article Synopsis

  • The study explores how von Willebrand factor (VWF) antigen levels are crucial in diagnosing acquired von Willebrand syndrome (AVWS) but can be influenced by underlying diseases.
  • A case of a 60-year-old AVWS patient with a monoclonal gammopathy showed normal VWF:Ag levels despite reduced VWF activity, revealing discrepancies in the test results.
  • The research indicates that conventional latex immunoassay (LIA) methods could overestimate VWF:Ag due to non-specific reactions, emphasizing the need for careful interpretation of VWF-Ag results in patients with AVWS.

Article Abstract

In the diagnosis and treatment of acquired von Willebrand syndrome (AVWS), von Willebrand factor (VWF) antigen levels (VWF:Ag) are helpful for quantifying blood VWF-protein levels. Most clinical laboratories measure VWF:Ag by latex immunoassay (LIA), but underlying diseases of AVWS may influence LIA results. A 60 year-old AVWS patient with immunoglobulin G (IgG) kappa-type monoclonal gammopathy of undetermined significance (MGUS) showed reduced VWF activity but normal levels of VWF:Ag. His VWF multimers were broadly decreased, which represented a large discrepancy with VWF:Ag. To investigate the mechanism of this discrepancy, we measured the patient's plasma VWF:Ag by in-house enzyme-linked immunosorbent assay (ELISA) and LIA. We also purified the IgG fraction from the patient's serum and measured VWF:Ag in VWF-deficient plasma supplemented with this fraction. VWF:Ag measured by in-house ELISA (VWF:Ag) was much lower than that measured by LIA (VWF:Ag), which indicated reduced VWF-protein volume in blood. Indeed, VWF:Ag was detected by LIA in VWF-deficient plasma spiked with a patient-derived IgG fraction. These results suggest that LIA detected a non-specific immunoreaction and overestimated the patient's VWF:Ag. Clinicians should be aware that underlying diseases of AVWS could influence the LIA system, and interpret VWF:Ag cautiously.

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Source
http://dx.doi.org/10.1007/s12185-024-03861-6DOI Listing

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