Disorders of Hemoglobin

Foreword; Introduction; 1. Historical aspects; Part I. Molecular, Cellular, and Genetic Basis of Hemoglobin Disorders: 2. Developmental erythropoiesis; 3. Erythropoiesis; 4. Nuclear factors that regulate erythropoiesis; 5. Organization, evolution and regulation of the globin genes; 6. Molecular genetics of human globin genes; 7. Molecular and cellular basis of hemoglobin switching; 8. Hemoglobin synthesis and translational control; 9. Hemoglobin structure and function; 10. Hemoglobins of the embryo and fetus and minor hemoglobins of adults; Part II. The b Thalassemias: 11. Pathophysiology of b thalassemia; 12. Molecular mechanisms of b thalassemia; 13. Clinical aspects of b thalassemia; 14. Structural variants with a b thalassemic phenotype; 15 Deletion HPFH and the delta-b thalassemias; Part III. The a Thalassemias: 16. Pathophysiology of the a thalassemias; 17. Molecular mechanisms of a thalassemia; 18. Clinical and laboratory features of the a thalassemia syndromes; 19. a Thalassemia mental retardation syndromes; Part IV. Sickle Cell Disease: 20. Pathophysiology of sickle cell anemia; 21. Red cell membrane in sickle cell disease; 22. Cellular adherence in sickle cell disease; 23. Sickle hemoglobin polymerization; 24. Clinical aspects of sickle cell anemia in adults and children; 25. The nature and treatment of the acute pain episode; 26. Genetic variability in sickle cell anemia; 27. Hb SC and Hb C disease; 28. Other sickle hemoglobinopathies; 29. Sickle cell trait; Part V. Epidemiology and Genetic Selection of Hemoglobinopathies and Thalassemia: 30. Balanced polymorphism in sickle cell disease and thalassemia-malaria; 31. Worldwide distribution of b thalassemia; 32. Worldwide distribution of a thalassemia; 33. Geographic heterogeneity of sickle cell anemia; Part VI. Diagnosis and Special Treatments for Sickle Cell Anemia and b Thalassemia Introduction: 34. Laboratory diagnosis, animal models; 35. DNA-based diagnosis of hemoglobin disorders; 36. Antenatal diagnosis, neonatal screening, population screening; 37. Transfusion and iron chelation in sickle cell disease and thalassemia; 38. Pharmacologic treatment of sickle cell disease and thalassemia; 39. Bone marrow transplantation in b thalassemia; 40. Bone marrow transplantation in sickle cell anemia; 41. Prospects for gene therapy in sickle cell disease and thalassemia; 42. Experimental treatment of sickle cell anemia and thalassemia; Part VII. Other Inherited Disorders: 43. Hb E disorders; 44. Unstable hemoglobins, hemoglobins with altered O2 affinity, Hb M; 45. Other mutant hemoglobins; Part VIII. Acquired Disorders of Hemoglobin: 46. Acquired and secondary disorders of hemoglobin.