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Circulating endothelial cells and progenitors as prognostic factors during autoimmune thrombotic thrombocytopenic purpura: results of a prospective multicenter French study.
Widemann, A; Pasero, C; Arnaud, L; Poullin, P; Loundou, A D; Choukroun, G; Sanderson, F; Lacroix, R; Sabatier, F; Coppo, P; Dignat-George, F; Kaplanski, G.
Affiliation
  • Widemann A; Vascular Research Center of Marseille, Inserm, UMRS_1076, Aix-Marseille Université, Marseille, France.
J Thromb Haemost ; 12(10): 1601-9, 2014 Oct.
Article in En | MEDLINE | ID: mdl-25088020
ABSTRACT

BACKGROUND:

Autoimmune thrombotic thrombocytopenic purpura (AI-TTP) is characterized by an excess of circulating ultralarge von Willebrand factor (VWF) caused by anti-ADAMTS-13 autoantibodies. 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 (CECs), soluble P-selectin (sP-selectin), or VWF, and of endothelial repair, such as circulating progenitor cells (CPCs) and endothelial progenitor cells (EPCs), in AI-TTP, in relation to disease severity and prognosis.

RESULTS:

Twenty-two patients were included in this study. CEC (P < 0.01), VWF (P < 0.05) and sP-selectin (P < 0.01) levels were significantly increased during crisis, and returned to baseline levels during remission. Both CEC (P < 0.05) and sP-selectin (P < 0.05) levels were significantly higher in patients who died or developed neurologic sequelae. CPC levels were substantially increased during the acute phase of the disease (P < 0.001), and returned to baseline levels during remission. Among CPCs, EPC levels were also increased during crisis (P < 0.05) and significantly decreased during remission. Patients who received < 16 plasma exchanges (PEs) had significantly higher EPC counts (P < 0.05) than those who needed more numerous PEs 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 CECs and EPCs may be promising prognostic biomarkers of the disease.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Purpura, Thrombotic Thrombocytopenic / Autoimmune Diseases / Stem Cells / Endothelial Cells Type of study: Clinical_trials / Observational_studies / Prognostic_studies / Risk_factors_studies Limits: Adult / Aged / Female / Humans / Male / Middle aged Country/Region as subject: Europa Language: En Journal: J Thromb Haemost Journal subject: HEMATOLOGIA Year: 2014 Document type: Article Affiliation country: Francia

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Purpura, Thrombotic Thrombocytopenic / Autoimmune Diseases / Stem Cells / Endothelial Cells Type of study: Clinical_trials / Observational_studies / Prognostic_studies / Risk_factors_studies Limits: Adult / Aged / Female / Humans / Male / Middle aged Country/Region as subject: Europa Language: En Journal: J Thromb Haemost Journal subject: HEMATOLOGIA Year: 2014 Document type: Article Affiliation country: Francia