Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis

Polycythemia vera (PV) causes thrombosis. Erythrocytosis and cell adhesiveness are responsible for thrombosis. JAK2V617F causes inflammation and autoimmunity; however, whether or not autoimmunity or inflammation causes thrombosis has yet to be proven. In 60 PV patients, we analyzed JAK2V671F and its...

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Main Authors: Rossella Cacciola, Elio Gentilini Cacciola, Veronica Vecchio, Emma Cacciola
Format: Article
Language:English
Published: MDPI AG 2022-04-01
Series:Diagnostics
Subjects:
Online Access:https://www.mdpi.com/2075-4418/12/5/1077
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author Rossella Cacciola
Elio Gentilini Cacciola
Veronica Vecchio
Emma Cacciola
author_facet Rossella Cacciola
Elio Gentilini Cacciola
Veronica Vecchio
Emma Cacciola
author_sort Rossella Cacciola
collection DOAJ
description Polycythemia vera (PV) causes thrombosis. Erythrocytosis and cell adhesiveness are responsible for thrombosis. JAK2V617F causes inflammation and autoimmunity; however, whether or not autoimmunity or inflammation causes thrombosis has yet to be proven. In 60 PV patients, we analyzed JAK2V671F and its allele burden, autoimmune Th17 cells, interleukin-17 (IL-17), anti-endothelial cell antibodies (AECAs), endothelial leukocyte adhesion molecule-1 (ELAM-1), intercellular adhesion molecule-1 (ICAM-1), and von Willebrand factor antigen (VWF: Ag). Fifty blood donors were used as the controls. All patients were on phlebotomy-maintaining hematocrit <45% and aspirin. Of the 60 patients, 40 had thrombosis. Those patients with thrombosis had a higher JAK2V617F allele burden than those without thrombosis, andTh17 cells and IL-17 were also higher in patients with thrombosis. Interestingly, we observed a high AECA IgG ELISA ratio (ER) in patients with thrombosis, which was normal in patients without thrombosis. We found high ELAM-1 and ICAM-1 as well as high VWF:Ag in patients with thrombosis compared to patients without thrombosis. AECA-positive sera from patients with thrombosis showed enhanced binding to cytokine-treated HUVEC and a positive antibody-dependent cellular cytotoxicity, suggesting that AECA may contribute to vascular injury. A positive correlation between AECAs, allele burden, and thrombosis was found. These results suggest that autoimmunity may be an additional mechanism in PV thrombogenesis.
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spelling doaj.art-fdf329a015e44444a3bdccfc8543b5152023-11-23T10:39:00ZengMDPI AGDiagnostics2075-44182022-04-01125107710.3390/diagnostics12051077Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and ThrombosisRossella Cacciola0Elio Gentilini Cacciola1Veronica Vecchio2Emma Cacciola3Hemostasis Unit, Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, ItalyPoliclinico “Umberto I”, Department of Public Health and Infectious Diseases, “Sapienza” University of Rome, 00182 Rome, ItalyHemostasis Unit, Medical School of Catania, University of Catania, 95123 Catania, ItalyHemostasis Unit, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, 95123 Catania, ItalyPolycythemia vera (PV) causes thrombosis. Erythrocytosis and cell adhesiveness are responsible for thrombosis. JAK2V617F causes inflammation and autoimmunity; however, whether or not autoimmunity or inflammation causes thrombosis has yet to be proven. In 60 PV patients, we analyzed JAK2V671F and its allele burden, autoimmune Th17 cells, interleukin-17 (IL-17), anti-endothelial cell antibodies (AECAs), endothelial leukocyte adhesion molecule-1 (ELAM-1), intercellular adhesion molecule-1 (ICAM-1), and von Willebrand factor antigen (VWF: Ag). Fifty blood donors were used as the controls. All patients were on phlebotomy-maintaining hematocrit <45% and aspirin. Of the 60 patients, 40 had thrombosis. Those patients with thrombosis had a higher JAK2V617F allele burden than those without thrombosis, andTh17 cells and IL-17 were also higher in patients with thrombosis. Interestingly, we observed a high AECA IgG ELISA ratio (ER) in patients with thrombosis, which was normal in patients without thrombosis. We found high ELAM-1 and ICAM-1 as well as high VWF:Ag in patients with thrombosis compared to patients without thrombosis. AECA-positive sera from patients with thrombosis showed enhanced binding to cytokine-treated HUVEC and a positive antibody-dependent cellular cytotoxicity, suggesting that AECA may contribute to vascular injury. A positive correlation between AECAs, allele burden, and thrombosis was found. These results suggest that autoimmunity may be an additional mechanism in PV thrombogenesis.https://www.mdpi.com/2075-4418/12/5/1077polycythemia veraJAK2V617F allele burdenAECAthrombosis
spellingShingle Rossella Cacciola
Elio Gentilini Cacciola
Veronica Vecchio
Emma Cacciola
Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis
Diagnostics
polycythemia vera
JAK2V617F allele burden
AECA
thrombosis
title Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis
title_full Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis
title_fullStr Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis
title_full_unstemmed Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis
title_short Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis
title_sort impact of anti endothelial cell antibodies aecas in patients with polycythemia vera and thrombosis
topic polycythemia vera
JAK2V617F allele burden
AECA
thrombosis
url https://www.mdpi.com/2075-4418/12/5/1077
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