Nabil Tabish: The Paradox of Zero – Investigating Extreme VWF Discordance
Nabil Tabish, Regional Medical Director at Kaiser Permanente, shared a post on LinkedIn:
“The infographic highlights the investigation of extreme VWF discordance, specifically when a patient presents with a severe qualitative defect yielding a VWF activity/Ag ratio of 0.00.
It emphasizes that clinicians must first rule out potential assay failures, such as rheumatoid factor or paraprotein interference, before confirming a true diagnosis.
It details the primary causes of Acquired von Willebrand Syndrome (AVWS), illustrating pathophysiological mechanisms like antibody-mediated clearance, high-shear proteolysis, and tumor cell adsorption.
It identifies lymphoproliferative disorders as the most likely mechanism causing such a severe, near-zero VWF activity defect in elderly patients.
Lastly, it demonstrates how an elevated FVIII level helps confirm the defect is isolated to specific VWF domains by completely ruling out conditions like Type 2N VWD and acquired hemophilia A.”
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