Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19

Defibrotide, which is approved for treating hepatic veno-occlusive disease (VOD)/sinusoidal obstruction syndrome (SOS), exhibits pleiotropic anti-inflammatory, anti-thrombotic, and fibrinolytic properties, conferring broad endothelial protective effects. Given these mechanisms, defibrotide has poten...

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Main Authors: Edward Richardson, Clifton C. Mo, Eleonora Calabretta, Francesco Corrado, Mehmet H. Kocoglu, Rebecca M. Baron, Jean Marie Connors, Massimo Iacobelli, Lee-Jen Wei, Emily J. Benjamin, Aaron P. Rapoport, Maribel Díaz-Ricart, Antonio José Martínez-Mellado, Carmelo Carlo-Stella, Paul G. Richardson, José M. Moraleda
Format: Article
Language:English
Published: MDPI AG 2025-07-01
Series:Biomolecules
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Online Access:https://www.mdpi.com/2218-273X/15/7/1004
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author Edward Richardson
Clifton C. Mo
Eleonora Calabretta
Francesco Corrado
Mehmet H. Kocoglu
Rebecca M. Baron
Jean Marie Connors
Massimo Iacobelli
Lee-Jen Wei
Emily J. Benjamin
Aaron P. Rapoport
Maribel Díaz-Ricart
Antonio José Martínez-Mellado
Carmelo Carlo-Stella
Paul G. Richardson
José M. Moraleda
author_facet Edward Richardson
Clifton C. Mo
Eleonora Calabretta
Francesco Corrado
Mehmet H. Kocoglu
Rebecca M. Baron
Jean Marie Connors
Massimo Iacobelli
Lee-Jen Wei
Emily J. Benjamin
Aaron P. Rapoport
Maribel Díaz-Ricart
Antonio José Martínez-Mellado
Carmelo Carlo-Stella
Paul G. Richardson
José M. Moraleda
author_sort Edward Richardson
collection DOAJ
description Defibrotide, which is approved for treating hepatic veno-occlusive disease (VOD)/sinusoidal obstruction syndrome (SOS), exhibits pleiotropic anti-inflammatory, anti-thrombotic, and fibrinolytic properties, conferring broad endothelial protective effects. Given these mechanisms, defibrotide has potential utility in various conditions involving endothelial injury or activation. In this review we outline the endothelial-protective mechanisms of defibrotide and comprehensively summarize current evidence supporting its applications in hematologic malignancies, including the prevention and treatment of hepatic VOD/SOS, graft-versus-host disease, and transplant-associated thrombotic microangiopathy. Additionally, we discuss its role in mitigating key toxicities linked to chimeric antigen receptor (CAR) T-cell therapies and bispecific antibodies, such as cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). We also explore emerging evidence on defibrotide’s potential in SARS-CoV-2 infection-associated endotheliopathies, including acute COVID-19 and post-acute sequelae of SARS-CoV-2 infection (“long-COVID”), and the endothelial protective activity of defibrotide in these settings. Finally, we highlight potential future applications of defibrotide in hematologic malignancies and viral infections, emphasizing its multimodal mechanism of action.
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spelling doaj-art-629ed4361b3d4353b86caaa6e85e5b712025-08-20T03:08:05ZengMDPI AGBiomolecules2218-273X2025-07-01157100410.3390/biom15071004Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19Edward Richardson0Clifton C. Mo1Eleonora Calabretta2Francesco Corrado3Mehmet H. Kocoglu4Rebecca M. Baron5Jean Marie Connors6Massimo Iacobelli7Lee-Jen Wei8Emily J. Benjamin9Aaron P. Rapoport10Maribel Díaz-Ricart11Antonio José Martínez-Mellado12Carmelo Carlo-Stella13Paul G. Richardson14José M. Moraleda15Department of Medicine, Warren Alpert Medical School at Brown University, Providence, RI 02903, USADepartment of Medical Oncology, Dana-Farber Cancer Institute, Jerome Lipper Center for Multiple Myeloma Research, Harvard Medical School, Boston, MA 02215, USADepartment of Biomedical Sciences, Humanitas University, 20089 Milan, ItalyDepartment of Medical Oncology, Dana-Farber Cancer Institute, Jerome Lipper Center for Multiple Myeloma Research, Harvard Medical School, Boston, MA 02215, USAUniversity of Maryland Greenebaum Comprehensive Cancer Center, Department of Medicine, University of Maryland School of Medicine, Baltimore, MD 21201, USADivision of Pulmonary and Critical Care Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA 02115, USADivision of Hematology, Brigham and Women’s Hospital, Boston, MA 02115, USATechitra S.r.l., 20123 Milan, ItalyDepartment of Biostatistics, Harvard T.H. Chan School of Public Health, Boston, MA 02115, USADepartment of Medical Oncology, Dana-Farber Cancer Institute, Jerome Lipper Center for Multiple Myeloma Research, Harvard Medical School, Boston, MA 02215, USAUniversity of Maryland Greenebaum Comprehensive Cancer Center, Department of Medicine, University of Maryland School of Medicine, Baltimore, MD 21201, USAHematopathology, Pathology Department, CDB, Hospital Clinic, IDIBAPS, 08036 Barcelona, SpainDepartment of Hematology, University Hospital Virgen de la Arrixaca, IMIB-Pascual Parrilla, University of Murcia, 30120 Murcia, SpainDepartment of Biomedical Sciences, Humanitas University, 20089 Milan, ItalyDepartment of Medical Oncology, Dana-Farber Cancer Institute, Jerome Lipper Center for Multiple Myeloma Research, Harvard Medical School, Boston, MA 02215, USADepartment of Medicine, Faculty of Medicine, Institute of Biomedical Research (IMIB-Pascual Parrilla), University of Murcia, 30120 Murcia, SpainDefibrotide, which is approved for treating hepatic veno-occlusive disease (VOD)/sinusoidal obstruction syndrome (SOS), exhibits pleiotropic anti-inflammatory, anti-thrombotic, and fibrinolytic properties, conferring broad endothelial protective effects. Given these mechanisms, defibrotide has potential utility in various conditions involving endothelial injury or activation. In this review we outline the endothelial-protective mechanisms of defibrotide and comprehensively summarize current evidence supporting its applications in hematologic malignancies, including the prevention and treatment of hepatic VOD/SOS, graft-versus-host disease, and transplant-associated thrombotic microangiopathy. Additionally, we discuss its role in mitigating key toxicities linked to chimeric antigen receptor (CAR) T-cell therapies and bispecific antibodies, such as cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). We also explore emerging evidence on defibrotide’s potential in SARS-CoV-2 infection-associated endotheliopathies, including acute COVID-19 and post-acute sequelae of SARS-CoV-2 infection (“long-COVID”), and the endothelial protective activity of defibrotide in these settings. Finally, we highlight potential future applications of defibrotide in hematologic malignancies and viral infections, emphasizing its multimodal mechanism of action.https://www.mdpi.com/2218-273X/15/7/1004CAR T-cell therapybispecific antibodies/bispecific T-cell engagersCOVID-19CRSendotheliitisendotheliopathy
spellingShingle Edward Richardson
Clifton C. Mo
Eleonora Calabretta
Francesco Corrado
Mehmet H. Kocoglu
Rebecca M. Baron
Jean Marie Connors
Massimo Iacobelli
Lee-Jen Wei
Emily J. Benjamin
Aaron P. Rapoport
Maribel Díaz-Ricart
Antonio José Martínez-Mellado
Carmelo Carlo-Stella
Paul G. Richardson
José M. Moraleda
Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19
Biomolecules
CAR T-cell therapy
bispecific antibodies/bispecific T-cell engagers
COVID-19
CRS
endotheliitis
endotheliopathy
title Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19
title_full Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19
title_fullStr Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19
title_full_unstemmed Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19
title_short Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19
title_sort defibrotide for protecting against and managing endothelial injury in hematologic malignancies and covid 19
topic CAR T-cell therapy
bispecific antibodies/bispecific T-cell engagers
COVID-19
CRS
endotheliitis
endotheliopathy
url https://www.mdpi.com/2218-273X/15/7/1004
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