Inserm, Institut national de la santé et de la recherche médicale
Faculté de pharmacie, Aix Marseille Université

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Circulating endothelial cells and progenitors as prognosis factors during auto-immune thrombotic thrombocytopenic purpura : Results of a prospective multicenter french study.

Widemann A, Pasero C, Arnaud L, Poullin P, Loundou AD, Choukroun G, Sanderson F, Lacroix R, Sabatier F, Coppo P, Dignat-George F, Kaplanski G ; The ENDO-13 study group
J Thromb Haemost. 2014 Aug 2

Autoimmune thrombotic thrombocytopenic purpura (AI-TTP) is characterized by an excess of circulating ultra-large von Willebrand Factor (vWF) due to anti-ADAMTS13 auto-antibodies. Animal studies however have shown that endothelial cell activation may also be an important trigger of AI-TTP.
To prospectively study circulating biomarkers of endothelial lesion and activation such as circulating endothelial cells (CEC), soluble (s)P-selectin or vWF and of endothelial repair such as circulating progenitor cells (CPC) and endothelial progenitor cells (EPC) in AI-TTP, in correlation with disease severity and prognosis.
22 patients were included in this study. CEC (p<0.01), vWF (p<0.05) and sP-selectin (p<0,01) were significantly increased during crisis and returned to baseline levels during remission. Both CEC (p<0.05) and sP-selectin (p<0.05) were significantly higher in patients who died or developed neurological sequelae. CPC counts were highly increased during the acute phase of the disease (p<0.001) and returned to baseline levels during remission. Among CPC, EPC were also increased during crisis (p<0.05) and significantly decreased during remission. Patients who received less than 16 plasma exchange (PE) therapy had significantly higher EPC counts (p<0.05) than those who needed more numerous PE to obtain remission, suggesting that initial EPC counts may be associated with faster endothelial repair.
The profile of circulating endothelial markers shows massive endothelial activation and repair/remodeling during AI-TTP, and suggests that CEC and EPC may be promising prognosis biomarkers of the disease. This article is protected by copyright. All rights reserved.