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

BACKGROUND :
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.
OBJECTIVES :
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.
RESULTS :
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.
CONCLUSION :
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.