Alan Nurden: Angiodysplasia and Gastrointestinal Bleeding in Von Willebrand Disease
Alan Nurden, Emeritus Research Director at CNRS, Co-Founder of the French National Reference Centre for Inherited Platelet Disorders (CRPP), shared on LinkedIn about a recent article by Adela Constantinescu-Bercu et al, published in Blood:
“This online paper attacks an important problem, angiodysplasia and gastrointestinal (GI) bleeding in von Willebrand disease. The authors show a role for VWF in the secretion of angiopoietin-2 (angpt-2) from endothelial cells and alterations in the angpt-1/2 ratio at lesion sites in VWD-/- mice. Significantly, inhibition of angpt-2 normalized vascular remodeling in the mice.
From a personal point of view, I would like to see a similar study in the inherited platelet disorder, Glanzmann thrombasthenia (GT), where GI bleeding is also a terrible problem for a subset of patients. Platelets secrete angpt-1 and its role is considered anti-inflammatory.
It would be interesting to measure the angpt-1/2 ratio in GT patients with nor without angiodysplasia and to compare those whose gene defects affect ITGA2B (and are restricted to alphaIIbbeta3 in platelets), to those whose gene defects affect ITGB3 and extend to alphavbeta3, a purported target of angpt-2 in endothelial cells.”
Title: Von Willebrand Factor Deficiency Impairs Angiogenesis via Angiopoietin-2: Relevance for Gut Angiodysplasia
Authors: Adela Constantinescu-Bercu, Koval E Smith, Shuo Yi Wong, Mattia Ballerini, Alessia Nastro, Benjamin G Wiggins, Daniela Pirri, Yucheng Li, Juun Anora Maria Evers, Olga Tsiamita, Matthew Dibble, Charis Pericleous, Koralia E Paschalaki, Graeme M Birdsey, Jeremiah Bernier-Latmani, Tatiana V Petrova, Mike A Laffan, Suthesh Sivapalaratnam, Marco Rasponi, Anna M. Randi
Read the Full Article on Blood.
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