Description
Springer Aplastic Anemia and Other Bone Marrow Failure Syndromes 1st Editon 2011 Softbound by Nasrollah T. Shahidi
During the past decade, there have been numerous direct and indirect scientific contributions to both the etiology and therapy of aplastic anemia and related bone marrow failure syndromes. Clinical observations, such as autologous bone marrow recovery after conditioning with immunosup pressive agents for bone marrow transplantation; failure to achieve en graftment in some identical twins without prior immunosuppressive ther apy; and hematologic response to immunosuppressive agents, have led to the concept of immune-mediated etiology of acquired aplastic anemia. Such a concept was further strengthened by laboratory findings, implicat ing the role of activated cytotoxic T lymphocytes and abnormal produc tion of inhibitory lymphokines. The immunologic mechanisms may also apply to the idiosyncratic bone marrow aplasias associated with drugs, toxic chemicals, and viruses. These agents may alter normal cellular recog nition sites by interacting with cellular components and result in loss of self tolerance. Immunologic mechanisms have long been advocated in many other organ failures, and the hemopoietic organ is no exception. It is of interest that parallel clinical and laboratory investigations in juvenile diabetes mellitus type I and in rodent models of this disease have yielded results compatible with the same pathogenic mechanisms. The infiltration of pancreatic islets by activated T lymphocytes, functional and morphological alterations of islet cells upon incubation with lymphokines such as gamma interferon and tumor necrosis factor, and clinical response to cyclosporine are a few examples. 1. Bone Marrow and Hemopoiesis.- 2. Acquired Aplastic Anemia: Classification and Etiologic Considerations.- 3. Constitutional Aplastic Anemia.- 4. Cytogenetics in Constitutional Aplastic Anemia.- 5. Cellular Interactions in the Suppression of Hematopoiesis.- 6. Flow Cytofluorometric Analysis of Patient Mononuclear Cells and Clinical Response to Antithymocyte Globulin.- 7. Prospects for the Treatment of Aplastic Anemia.- 8. Treatment of Severe Aplastic Anemia: A 12-Year Follow-Up of Patients after Bone Marrow Transplantation or after Therapy with Antilymphocyte Globulin.- 9. Long-Term Comparison of Immunosuppressive Therapy with Antithymocyte Globulin to Bone Marrow Transplantation in Aplastic Anemia.- 10. Treatment of Severe Aplastic Anemia (SAA) with Bone Marrow Transplantation (BMT) or Immunosuppression: A Report of the EBMT SAA Working Party.- 11. Treatment of Aplastic Anemia: Bone Marrow Transplantation, Immunomodulatory Therapy, and Hematopoietic Growth Factors.- 12. Bone Marrow Transplantation for Fanconi Anemia.- 13. The Use of Etiocholanolone and Antithymus Globulin to Treat Aplastic Anemia: An Uncontrolled Comparative Study.- 14. Treatment of Acquired Aplastic Anemia with Cyclosporine and Androgens.- 15. Congenital Hypoplastic Anemia (Diamond-Blackfan Anemia).- 16. Pure Red Cell Aplasia.- 17. The Preleukemic Syndrome (Hematopoietic Dysplasia).- 18. Summary and Conclusions.