This issue of Hematology/Oncology Clinics, guest edited by Dr. Elliott Vichinsky, is devoted to Sickle Cell Disease, and focuses on pathophysiology of hemoglobinopathies, therapeutic targets, and new approaches to correcting ineffective erythropoiesis and iron dysregulation. Articles in this issue include Polymerization and red cell membrane changes; Overview on reperfusion injury in the pathophysiology of SCD; Regulation of ineffective erythropoiesis in iron metabolism; Altering oxygen affinity; Cellular adhesion and the endothelium; Arginine therapy; Role of the hemostatic system on SCD pathophysiology and potential therapeutics; Adenosine signaling and novel therapies; New approaches to correcting ineffective erythropoiesis and iron dysregulation; New approaches to correcting ineffective erythropoiesis and iron dysregulation; Fetal hemoglobin induction; Gene therapy for hemoglobinopathies; and Oxidative injury and the role of antioxidant therapy.
1. Cover image - p. 1 2. Title page - p. 2 3. Table of Contents - p. 3 4. Contributors - p. 4 5. Forthcoming Issues - p. 7 6. Preface: Emerging Therapy in Hemoglobinopathies: Lessons from the Past and Optimism for the Future - p. 8 7. Dedication - p. 12 8. Chapter 1: Hemoglobin S Polymerization and Red Cell Membrane Changes - p. 13 9. Chapter 2: Ischemia-reperfusion Injury in Sickle Cell Anemia - p. 27 10. Chapter 3: Gene Therapy for Hemoglobinopathies - p. 37 11. Chapter 4: Therapeutic Strategies to Alter the Oxygen Affinity of Sickle Hemoglobin - p. 46 12. Chapter 5: Targeted Fetal Hemoglobin Induction for Treatment of Beta Hemoglobinopathies - p. 55 13. Chapter 6: Does Erythropoietin Have a Role in the Treatment of β-Hemoglobinopathies? - p. 65 14. Chapter 7: Inflammatory Mediators of Endothelial Injury in Sickle Cell Disease - p. 74 15. Chapter 8: The Role of Adenosine Signaling in Sickle Cell Therapeutics - p. 86 16. Chapter 9: Alterations of the Arginine Metabolome in Sickle Cell Disease - p. 93 17. Chapter 10: Cellular Adhesion and the Endothelium - p. 105 18. Chapter 11: Cellular Adhesion and the Endothelium - p. 116 19. Chapter 12: Role of the Hemostatic System on Sickle Cell Disease Pathophysiology and Potential Therapeutics - p. 124 20. Chapter 13: Modulators of Erythropoiesis - p. 136 21. Chapter 14: Modulation of Hepcidin as Therapy for Primary and Secondary Iron Overload Disorders - p. 143 22. Index - p. 152
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This issue of Hematology/Oncology Clinics, guest edited by Dr. Elliott Vichinsky, is devoted to Sickle Cell Disease, and focuses on pathophysiology of hemoglobinopathies, therapeutic targets, and new approaches to correcting ineffective erythropoiesis and iron dysregulation. Articles in this issue include Polymerization and red cell membrane changes; Overview on reperfusion injury in the pathophysiology of SCD; Regulation of ineffective erythropoiesis in iron metabolism; Altering oxygen affinity; Cellular adhesion and the endothelium; Arginine therapy; Role of the hemostatic system on SCD pathophysiology and potential therapeutics; Adenosine signaling and novel therapies; New approaches to correcting ineffective erythropoiesis and iron dysregulation; New approaches to correcting ineffective erythropoiesis and iron dysregulation; Fetal hemoglobin induction; Gene therapy for hemoglobinopathies; and Oxidative injury and the role of antioxidant therapy.
1. Cover image - p. 1 2. Title page - p. 2 3. Table of Contents - p. 3 4. Contributors - p. 4 5. Forthcoming Issues - p. 7 6. Preface: Emerging Therapy in Hemoglobinopathies: Lessons from the Past and Optimism for the Future - p. 8 7. Dedication - p. 12 8. Chapter 1: Hemoglobin S Polymerization and Red Cell Membrane Changes - p. 13 9. Chapter 2: Ischemia-reperfusion Injury in Sickle Cell Anemia - p. 27 10. Chapter 3: Gene Therapy for Hemoglobinopathies - p. 37 11. Chapter 4: Therapeutic Strategies to Alter the Oxygen Affinity of Sickle Hemoglobin - p. 46 12. Chapter 5: Targeted Fetal Hemoglobin Induction for Treatment of Beta Hemoglobinopathies - p. 55 13. Chapter 6: Does Erythropoietin Have a Role in the Treatment of β-Hemoglobinopathies? - p. 65 14. Chapter 7: Inflammatory Mediators of Endothelial Injury in Sickle Cell Disease - p. 74 15. Chapter 8: The Role of Adenosine Signaling in Sickle Cell Therapeutics - p. 86 16. Chapter 9: Alterations of the Arginine Metabolome in Sickle Cell Disease - p. 93 17. Chapter 10: Cellular Adhesion and the Endothelium - p. 105 18. Chapter 11: Cellular Adhesion and the Endothelium - p. 116 19. Chapter 12: Role of the Hemostatic System on Sickle Cell Disease Pathophysiology and Potential Therapeutics - p. 124 20. Chapter 13: Modulators of Erythropoiesis - p. 136 21. Chapter 14: Modulation of Hepcidin as Therapy for Primary and Secondary Iron Overload Disorders - p. 143 22. Index - p. 152
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