In recent years, many advances have been made in the better understanding of endothelial cell’s function. In the field of hematology, especially, endothelial injury is a hallmark not only in benign hematological disorders, such as sickle cell disease and thrombotic thrombocytopenic purpura (TTP) but also observed in clinical syndromes complicating hematopoietic stem cell transplantation (HSCT), which is considered the only curative option for various malignant and benign clinical entities. In Endothelial injury syndromes following HSCT, transplant-associated thrombotic microangiopathy (TA-TMA), hepatic sinusoidal obstruction syndrome/veno-occlusive disease, and lung injury syndromes are included. Chimeric antigen receptor-T (CAR-T) cell immunotherapies, a cornerstone nowadays in the management of patients with relapsed/ refractory B-cell malignancies, might be complicated by endothelial injury. Specifically, cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome (ICANS), observed post-CAR-T cell infusion have been attributed to endothelial cells’ activation and injury. More data regarding markers of endothelial injury and activation in these clinical entities are crucial.
Innate immunity, including complement system and toll-like receptors, was initially considered as a first line of defense against pathogens. However, dysregulation of these systems has been implicated in the pathogenesis of several diseases. Increased complement system activation has been identified in disorders such as atypical hemolytic uremic syndrome, paroxysmal nocturnal hemoglobinuria, and TA-TMA. Based on these findings, safe and efficient complement inhibitors have been developed and widely used in clinical practice for the management of our patients. Furthermore, other signaling molecules of innate immunity, for example, toll-like receptors, have been studied as potential pathogenetic factors in myeloid malignancies, such as myelodysplastic syndromes.
An interplay between innate immunity, endothelial activation, and coagulation system activation has been reported in the above-described disorders. Disorders of coagulation can be presented with both hemorrhagic and thrombotic complications. A better understanding of the molecular mechanisms behind the “coagulopathies”, endothelium, and innate immunity is considered essential in order to develop personalized and efficient therapeutics for clinical entities such as hemophilia, antiphospholipid syndrome, disseminated intravascular coagulation, TTP, and thrombotic complications of HSCT. Coagulation system dysregulation is also of paramount importance to be investigated in patients with hematological malignancies.
This Research Topic is interested in publishing Original Research, Systematic Review, Methods, Review, Mini Review, Policy and Practice Reviews, Hypothesis and Theory, Perspective, Clinical Trial, Case Report, General Commentary, and Opinion articles. Basic, translational, and clinical research papers in the following topics are to be considered for publication. Topics may include (but are not limited to) studies in endothelial, innate immunity, and coagulation system dysregulation and activation in the following areas:
• Benign Hematological Disorders
• Hemoglobinopathies
• Hemophilia and other bleeding disorders
• Hematological Malignancies
• Immune Thrombocytopenia and Antiphospholipid Syndrome
• Hematopoietic Stem Cell Transplantation
• Chimeric antigen receptor-T (CAR-T) cell immunotherapies
In recent years, many advances have been made in the better understanding of endothelial cell’s function. In the field of hematology, especially, endothelial injury is a hallmark not only in benign hematological disorders, such as sickle cell disease and thrombotic thrombocytopenic purpura (TTP) but also observed in clinical syndromes complicating hematopoietic stem cell transplantation (HSCT), which is considered the only curative option for various malignant and benign clinical entities. In Endothelial injury syndromes following HSCT, transplant-associated thrombotic microangiopathy (TA-TMA), hepatic sinusoidal obstruction syndrome/veno-occlusive disease, and lung injury syndromes are included. Chimeric antigen receptor-T (CAR-T) cell immunotherapies, a cornerstone nowadays in the management of patients with relapsed/ refractory B-cell malignancies, might be complicated by endothelial injury. Specifically, cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome (ICANS), observed post-CAR-T cell infusion have been attributed to endothelial cells’ activation and injury. More data regarding markers of endothelial injury and activation in these clinical entities are crucial.
Innate immunity, including complement system and toll-like receptors, was initially considered as a first line of defense against pathogens. However, dysregulation of these systems has been implicated in the pathogenesis of several diseases. Increased complement system activation has been identified in disorders such as atypical hemolytic uremic syndrome, paroxysmal nocturnal hemoglobinuria, and TA-TMA. Based on these findings, safe and efficient complement inhibitors have been developed and widely used in clinical practice for the management of our patients. Furthermore, other signaling molecules of innate immunity, for example, toll-like receptors, have been studied as potential pathogenetic factors in myeloid malignancies, such as myelodysplastic syndromes.
An interplay between innate immunity, endothelial activation, and coagulation system activation has been reported in the above-described disorders. Disorders of coagulation can be presented with both hemorrhagic and thrombotic complications. A better understanding of the molecular mechanisms behind the “coagulopathies”, endothelium, and innate immunity is considered essential in order to develop personalized and efficient therapeutics for clinical entities such as hemophilia, antiphospholipid syndrome, disseminated intravascular coagulation, TTP, and thrombotic complications of HSCT. Coagulation system dysregulation is also of paramount importance to be investigated in patients with hematological malignancies.
This Research Topic is interested in publishing Original Research, Systematic Review, Methods, Review, Mini Review, Policy and Practice Reviews, Hypothesis and Theory, Perspective, Clinical Trial, Case Report, General Commentary, and Opinion articles. Basic, translational, and clinical research papers in the following topics are to be considered for publication. Topics may include (but are not limited to) studies in endothelial, innate immunity, and coagulation system dysregulation and activation in the following areas:
• Benign Hematological Disorders
• Hemoglobinopathies
• Hemophilia and other bleeding disorders
• Hematological Malignancies
• Immune Thrombocytopenia and Antiphospholipid Syndrome
• Hematopoietic Stem Cell Transplantation
• Chimeric antigen receptor-T (CAR-T) cell immunotherapies