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Disorders of Hemoglobin
Genetics, Pathophysiology, and Clinical Management

2nd Edition

$463.00 ( ) USD

H. Franklin Bunn, David Weatherall, Elaine Dzierzak, S. Philipsen, W. G. Wood, Bernard G. Forget, Ross C. Hardison, Gerd A. Blobel, Mitchell J. Weiss, George Stamatoyannopoulos, Daniel Kim-Shapiro, Martin H. Steinberg, Ronald L. Nagel, Dhananjat Kaul, Clint Joiner, Patrick Gallagher, Jane A. Leopold, Joseph Loscalzo, Gregory J. Kato, Mark T. Gladwin, Mary Fabry, Douglas R. Higgs, Vip Viprakasit, Don Bowden, Richard Gibbons, David Steensma, S. L. Thein, Nancy F. Olivieri, Suthat Fuchareon, Kwaku Ohene-Frempong, Matthew M. Heeney, Samir K. Ballas, James R. Eckman, Neeraj Agarwal, Thomas N. Williams, John M. Old, John Porter, Alan R. Cohen, Janet L. Kwiatkowski, Yogen Saunthararajah, George F. Atweh, Carlo Brugnara, Kirkwood A. Pritchard, Jr, Cheryl Hillery, Emanuele Angelucci, Mark Walters, Derek A. Persons, Brian P. Sorrentino, Arthur W. Nienhuis
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  • Date Published: September 2009
  • availability: This ISBN is for an eBook version which is distributed on our behalf by a third party.
  • format: Adobe eBook Reader
  • isbn: 9780511590276

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About the Authors
  • This book is a completely revised new edition of the definitive reference on disorders of hemoglobin. Authored by world-renowned experts, the book focuses on basic science aspects and clinical features of hemoglobinopathies, covering diagnosis, treatment, and future applications of current research. While the second edition continues to address the important molecular, cellular, and genetic components, coverage of clinical issues has been significantly expanded, and there is more practical emphasis on diagnosis and management throughout. The book opens with a review of the scientific underpinnings. Pathophysiology of common hemoglobin disorders is discussed next in an entirely new section devoted to vascular biology, the erythrocyte membrane, nitric oxide biology, and hemolysis. Four sections deal with α and β thalassemia, sickle cell disease, and related conditions, followed by special topics. The second edition concludes with current and developing approaches to treatment, incorporating new agents for iron chelation, methods to induce fetal hemoglobin production, novel treatment approaches, stem cell transplantation, and progress in gene therapy.

    • The definitive reference on disorders of hemoglobin
    • Authored by world renowned experts in this field
    • Provides practical approaches to treatment
    Read more

    Reviews & endorsements

    "This book is an excellent and truly extraordinary achievement of the editors and the authors. It is certainly a definitive reference work on the various disorders of hemoglobin. It is a must-have and a keepsake for any research scientist, professors seeking broader and deeper knowledge on the subject, or insight from the authors who are world-renowned experts. It is also a very valuable resource for any physician treating patients who have any disease relating to or resulting from hemoglobin disorders."
    Nano Khilnani, Biz India

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    Product details

    • Edition: 2nd Edition
    • Date Published: September 2009
    • format: Adobe eBook Reader
    • isbn: 9780511590276
    • contains: 121 b/w illus. 40 colour illus. 96 tables
    • availability: This ISBN is for an eBook version which is distributed on our behalf by a third party.
  • Table of Contents

    Foreword H. Franklin Bunn
    Preface
    Introduction David Weatherall
    Part I. The Cellular, Molecular and Genetic Basis of Hemoglobin Disorders: Introduction
    1. The development of hematopoiesis Elaine Dzierzak
    2. Erythropoiesis S. Philipsen and W. G. Wood
    3. The normal structure regulation of human globin gene clusters Bernard G. Forget and Ross C. Hardison
    4. The normal regulation of globin gene expression Gerd A. Blobel and Mitchell J. Weiss
    5. The molecular and cellular basis of developmental globin gene switching George Stamatoyannopoulos
    6. Structure and function of hemoglobin and its dysfunction in sickle cell disease Daniel Kim-Shapiro
    7. Hemoglobins of the embryo, fetus and adult Martin H. Steinberg and Ronald L. Nagel
    Part II. Pathophysiology of Hemoglobin and its Disorders: Introduction
    8. Vascular biology Dhananjat Kaul
    9. The erythrocyte membrane Clint Joiner and Patrick Gallagher
    10. The biology of vascular nitric oxide Jane A. Leopold and Joseph Loscalzo
    11. Mechanisms and complications of hemolysis in sickle cell disease and thalassemia Gregory J. Kato and Mark T. Gladwin
    12. The importance of animal models in understanding pathophysiology Mary Fabry
    Part III. α Thalassemia: Introduction:
    13. The molecular basis of alpha thalassemia Douglas R. Higgs and Vip Viprakasit
    14. The pathophysiology and clinical features of alpha thalassemia Douglas R. Higgs, Vip Viprakasit and Don Bowden
    15. Alpha thalassemia with mental retardation and myelodysplasia Douglas R. Higgs, Richard Gibbons, and David Steensma
    Part IV. Beta Thalassemia: Introduction:
    16. The molecular basis of beta thalassemia S. L. Thein and W. G. Wood
    17. Clinical aspects of beta thalassemia and related disorders Nancy F. Olivieri, D. K. Bowden, and D. J. Weatherall
    18. HbE disorders Suthat Fuchareon and David Weatherall
    Part V. Sickle Cell Disease: Introduction:
    19. Clinical and pathophysiological aspects of sickle cell anemia Martin Steinberg, Kwaku Ohene-Frempong, and Matthew M. Heeney
    20. Biology of pain and treatment of the sickle cell painful episode Samir K. Ballas and James R. Eckman
    21. HbSC disease and HbC Martin H. Steinberg and Ronald L. Nagel
    22. Sickle cell trait Martin H. Steinberg
    23. Other sickle hemoglobinopathies Martin H. Steinberg
    Part VI. Other Clinically Important Disorders of Hemoglobin: Introduction:
    24. Unstable hemoglobins
    hemoglobins with altered oxygen affinity
    hemoglobin M
    other variants of clinical and biological interest Martin H. Steinberg and Ronald L. Nagel
    25. Dyshemoglobinemias Neeraj Agarwal and Ronald L. Nagel
    Part VII. Special Topics in Hemoglobinopathies: Introduction:
    26. Population genetics and global health burden David J. Weatherall and Thomas N. Williams
    27. Genetic modulation of sickle cell disease and thalassemia Martin H. Steinberg and Ronald L. Nagel
    28. Developments in laboratory methods to detect hemoglobinopathies Mary Fabry and John M. Old
    Part VIII. New Approaches to the Treatment of Hemoglobinopathies and Thalassemia: Introduction:
    29. Transfusion and iron chelation John Porter, Alan R. Cohen and Janet L. Kwiatkowski
    30. Induction of HbF Yogen Saunthararajah and George F. Atweh
    31. Novel approaches to treatment - (anitoxidants, statins, anti-inflammatory agents, K-Cl and Gardos inhibitors, other transport inhibitors, NO) Carlo Brugnara, Kirkwood A. Pritchard, Jr and Cheryl Hillery
    32. Stem cell transplantation Emanuele Angelucci and Mark Walters
    33. Gene therapy Derek A. Persons, Brian P. Sorrentino, and Arthur W. Nienhuis.

  • Authors

    Martin H. Steinberg, Boston University
    Martin H. Steinberg is Professor of Medicine, Pediatrics, Pathology and Laboratory Medicine at Boston University School of Medicine. He is also Director at the Center of Excellence in Sickle Cell Disease, Boston Medical Center.

    Bernard G. Forget, Yale University, Connecticut
    Bernard G. Forget is Professor of Internal Medicine and Genetics at Yale University School of Medicine.

    Douglas R. Higgs, MRC Institute of Molecular Medicine, University of Oxford
    Douglas R. Higgs is Professor of Molecular Haematology at the University of Oxford.

    David J. Weatherall, University of Oxford
    David J. Weatherall is Regius Professor of Medicine Emeritus at the University of Oxford and Chancellor at Keele University.

    Contributors

    H. Franklin Bunn, David Weatherall, Elaine Dzierzak, S. Philipsen, W. G. Wood, Bernard G. Forget, Ross C. Hardison, Gerd A. Blobel, Mitchell J. Weiss, George Stamatoyannopoulos, Daniel Kim-Shapiro, Martin H. Steinberg, Ronald L. Nagel, Dhananjat Kaul, Clint Joiner, Patrick Gallagher, Jane A. Leopold, Joseph Loscalzo, Gregory J. Kato, Mark T. Gladwin, Mary Fabry, Douglas R. Higgs, Vip Viprakasit, Don Bowden, Richard Gibbons, David Steensma, S. L. Thein, Nancy F. Olivieri, Suthat Fuchareon, Kwaku Ohene-Frempong, Matthew M. Heeney, Samir K. Ballas, James R. Eckman, Neeraj Agarwal, Thomas N. Williams, John M. Old, John Porter, Alan R. Cohen, Janet L. Kwiatkowski, Yogen Saunthararajah, George F. Atweh, Carlo Brugnara, Kirkwood A. Pritchard, Jr, Cheryl Hillery, Emanuele Angelucci, Mark Walters, Derek A. Persons, Brian P. Sorrentino, Arthur W. Nienhuis

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