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Article

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

1
Hemostasis Unit, Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy
2
Policlinico “Umberto I”, Department of Public Health and Infectious Diseases, “Sapienza” University of Rome, 00182 Rome, Italy
3
Hemostasis Unit, Medical School of Catania, University of Catania, 95123 Catania, Italy
4
Hemostasis Unit, Department of Medical, Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, 95123 Catania, Italy
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work.
Academic Editor: Evangelos Terpos
Diagnostics 2022, 12(5), 1077; https://doi.org/10.3390/diagnostics12051077
Received: 27 March 2022 / Revised: 24 April 2022 / Accepted: 24 April 2022 / Published: 25 April 2022
(This article belongs to the Special Issue Diagnosis for Hematologic Disorders)
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. View Full-Text
Keywords: polycythemia vera; JAK2V617F allele burden; AECA; thrombosis polycythemia vera; JAK2V617F allele burden; AECA; thrombosis
MDPI and ACS Style

Cacciola, R.; Gentilini Cacciola, E.; Vecchio, V.; Cacciola, E. Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis. Diagnostics 2022, 12, 1077. https://doi.org/10.3390/diagnostics12051077

AMA Style

Cacciola R, Gentilini Cacciola E, Vecchio V, Cacciola E. Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis. Diagnostics. 2022; 12(5):1077. https://doi.org/10.3390/diagnostics12051077

Chicago/Turabian Style

Cacciola, Rossella, Elio Gentilini Cacciola, Veronica Vecchio, and Emma Cacciola. 2022. "Impact of Anti-Endothelial Cell Antibodies (AECAs) in Patients with Polycythemia Vera and Thrombosis" Diagnostics 12, no. 5: 1077. https://doi.org/10.3390/diagnostics12051077

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