Cooleys Anemia: Eighth Symposium, Volume 1054

Cooley′s anemia, or thalassemia major, is a blood disorder characterized by a marked increase in F hemoglobin and a decrease in the production of certain oxygen–carrying proteins in red blood cells.
Thalassemia major is the most severe form of the chronic familial anemias that result from the premature destruction of red blood cells and is inherited as an autosomal recessive trait. In the years since the Seventh Cooley′s Anemia Symposium, held in 1997, major advances have taken place in the understanding and treatment of the disease.
The molecular mechanisms responsible for the switch from fetal hemoglobin to adult hemoglobin production have been further clarified, while new drugs to enhance the production of fetal hemoglobin and relieve the anemia of thalassemia have been introduced and studied. Understanding of the relationship between molecular genotype and clinical phenotype has been advanced, and the techniques for molecular diagnosis, including prenatal diagnosis, have been vastly improved. Most importantly, there have been dramatic improvements in the treatment and prevention of complications of thalassemia. As a result, patients are now living longer, albeit with more disability.
Problems such as osteoporosis, heart failure, growth hormone deficiency, pulmonary hypertension, and infertility can now be detected early and treated. This volume not only focuses on the advances over the last six or seven years, but also illuminates many unsolved but critically important issues in the understanding and treatment of thalassemia, thus offering the scientific, clinical, care–giving, and patient communities the most up–to–date exchange on the current and future perspectives of the disease.
NOTE: Annals volumes are available for sale as individual books or as a journal. For information on institutional journal subscriptions, please visit www.blackwellpublishing.com/nyas.
ACADEMY MEMBERS: P lease contact the New York Academy of Sciences directly to place your order (www.nyas.org). Members of the New York Academy of Science receive full–text access to the Annals online and discounts on print volumes. Please visit www.nyas.org/membership/main.asp for more information about becoming a member.

Spis treści:
Preface (Elliott P. Vichinsky).
Thalassemia: The Continued Challenge (David G. Nathan).
Keynote Address: The Challenge of Thalassemia for the Developing Countries (David J. Weatherall).
Part I: The Natural History of Thalassemia Worldwide.
1. Changing Patterns of Thalassemia Worldwide (Elliott P. Vichinsky).
2. –Thalassemia: Hb H Disease and Hb Barts Hydrops Fetalis (David H.K. Chui).
3. Hemoglobin E–ß–Thalassemia: Progress Report from the International Study Group (Anuja Premawardhena, Shanthimala De Silver, Mahinda Arambepola, Nancy F. Olivieri, Elliott P. Vichinsky, Laura Merson, Giulia Muraco, Angela Allen, Christopher Fisher, Timothy Peto, and David J. Weatherall).
4. Survival and Complications in Thalassemia (C Borgna–Pignatti, M D Cappellini, P De Stefano, G C Del Vecchio, G L Forni, M R Gamberini, R Ghilardi, R Origa, A Piga, M A Romeo, H Zhao, and A Cnaan).
Part II: Gene Regulation and Therapy.
5. Role of Intergenic Human ––Globin Sequences in Human Hemoglobin Switching and Reactivation of Fetal Hemoglobin in Adult Erythroid Cells (Arthur Bank, David O′neill, Rocio Lopez, Dianne Pulte, Maureen Ward, Simon Mantha, and Christine Richardson).
6. Isolation and Characterization of Hematopoietic Transcription Factor Complexes by in Vivo Biotinylation Tagging and Mass Spectrometry (Frank Grosveld, Patrick Rodriguez, Natalia Meier, Sanja Krpic, Farzin Pourfarzad, Petros Papadopoulos, Katarzyna Kolodziej, George P. Patrinos, Arnd Hostert, and John Strouboulis).
7. Germline Epimutation: A Basis for Epigenetic Disease in Humans (David I.K. Martin, Robyn Ward, and Catherine M. Suter).
8. Progress Toward the Genetic Treatment of the ß–Thalassemias (Michel Sadelain, Leszek Lisowski, Selda Samakoglu, Stefano Rivella, Chad May, and Isabelle Riviere).
9. Understanding –Globin Gene Regulation: Aiming to Improve the Management of Thalassemia (D R Higgs, D Garrick, E Anguita, M De Gobbi, J Hughes, M Muers, D Vernimmen, K Lower, M Law, A Argentaro, M A Deville, and R Gibbons).
10. Role of Alpha Hemoglobin–Stabilizing Protein in Normal Erythropoiesis and ß–Thalassemia (Mitchell J. Weiss, Suiping Zhou, Liang Feng, David A. Gell, Joel P. Mackay, Yigong Shi, and Andrew J. Gow).
Part III: Mechanisms of Cell Injury from Iron Overload in Thalassemia.
11. Role of Iron in Inducing Oxidative Stress in Thalassemia: Can It Be Prevented by Inhibition of Absorption and by Antioxidants (Eliezer A. Rachmilewitz, Orly Weizer–Stern, Konstantin Adamsky, Ninette Amariglio, Gideon Rechavi, Laura Breda, Stefano Rivella, and Z Ioav Cabantchik).
12. Objectives and Mechanism of Iron Chelation Therapy (Chaim Hershko, Gabriela Link, Abraham M. Konijn, and Z Ioav Cabantchik).
13. Ferritin as an Iron Concentrator and Chelator Target (Xiaofeng Liu and Elizabeth C. Theil).
Part IV: Iron Chelator: Development and Therapy.
14. The Design of Orally Active Iron Chelators (Robert C. Hider and Tao Zhou).
15. Recent Insights into Interactions of Deferoxamine with Cellular and Plasma Iron Pools: Implications for Clinical Use (J B Porter, R Rafique, S Srichairatanakool, B A Davis, F T Shah, T Hair, and P Evans).
16. Deferiprone: New Insight (A Piga, S Roggero, T Vinciguerra, L Sacchetti, V Gallo, and F Longo).
17. Combined Therapy with Deferoxamine and Deferiprone (Antonis Kattamis).
18. Evaluation of ICL670, a Once–Daily Oral Iron Chelator in a Phase III Clinical Trial of ß–Thalassemia Patients with Transfusional Iron Overload (Renzo Galanello).
Part V: New Advances in Stem Cell Transplantation.
19. Unrelated Bone Marrow Transplantation for ß–Thalassemia Patients: The Experience of the Italian Bone Marrow Transplant Group (Giorgio La Nasa, Franca Argiolu, Claudio Giardini, Andrea Pession, Franca Fagioli, Giovanni Caocci, Adriana Vacca, Piero De Stefano, Eugenia Piras, Antonio Ledda, Antonio Piroddi, Roberto Littera, Sonia Nesci, and Franco Locatelli).
20. Bone Marrow Transplantation in Adults with Thalassemia: Treatment and Long–Term Follow–Up (Javid Gaziev, Pietro Sodani, Paola Polchi, Marco Andreani, and Guido Lucarelli).
21. Sibling Donor Cord Blood Transplantation for Thalassemia Major: Experience of the Sibling Donor Cord Blood Program (Mark C. Walters, Lynn Quirolo, Elizabeth T. Trachtenberg, Sandie Edwards, Lisa Hale, Joanna Lee, Joi Morton–Wiley, Keith Quirolo, Shanda Robertson, Julie Saba, and Bert Lubin).
22. Stem Cell Transplantation with S–59 Photochemically Treated T–Cell Add–Backs to Establish Allochimerism in Murine Thalassemia (Frans A. Kuypers, Gordon Watson, Ezra Sage, Mark C. Walters, James Hamrick, and John E. Hearst).
23. Preimplantation Genetics: Improving Access to Stem Cell Therapy (Anver Kuliev, Svetlana Rechitsky, Ilan Tur–Kaspa, and Yury Verlinsky).
Part VI: New Therapy for Thalassemia.
24. Pharmacological Induction of Fetal Hemoglobin: Why Haven′t We Been More Successful in Thalassemia (Hassana Fathallah, Millicent Sutton, and George F. Atweh).
25. Successful Correction of the Human Cooley′s Anemia ß–Thalassemia Major Phenotype Using a Lentiviral Vector Flanked by the Chicken Hypersensitive Site 4 Chromatin Insulator (Punam Malik, Paritha I. Arumugam, Jing–Kuan Yee, and Geetha Puthenveetil).
26. Single and Combination Drug Therapy for Fetal Hemoglobin Augmentation in Hemoglobin E–ß0–Thalassemia: Considerations