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Disorders of Hemoglobin Genetics, Pathophysiology, and Clinical Management

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ISBN-10: 0521875196

ISBN-13: 9780521875196

Edition: 2nd 2009

Authors: Martin H. Steinberg, Bernard G. Forget, Douglas R. Higgs, Ronald L. Nagel, David J. Weatherall

List price: $579.00
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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…    
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Book details

List price: $579.00
Edition: 2nd
Copyright year: 2009
Publisher: Cambridge University Press
Publication date: 8/17/2009
Binding: Hardcover
Pages: 846
Size: 8.50" wide x 10.00" long x 1.00" tall
Weight: 6.490
Language: English

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

Douglas R. Higgs is Professor of Molecular Haematology at the University of Oxford.

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

Foreword H. Franklin Bunn
Introduction David Weatherall
The Cellular, Molecular and Genetic Basis of Hemoglobin Disorders: Introduction
The development of hematopoiesis
The normal structure regulation of human globin gene clusters
The normal regulation of globin gene expression
The molecular and cellular basis of developmental globin gene switching
Structure and function of hemoglobin and its dysfunction in sickle cell disease
Hemoglobins of the embryo, fetus and adult
Pathophysiology of Hemoglobin and its Disorders: Introduction
Vascular biology
The erythrocyte membrane
The biology of vascular nitric oxide
Mechanisms and complications of hemolysis in sickle cell disease and thalassemia
The importance of animal models in understanding pathophysiology
+� Thalassemia: Introduction
The molecular basis of alpha thalassemia
The pathophysiology and clinical features of alpha thalassemia
Alpha thalassemia with mental retardation and myelodysplasia
Beta Thalassemia: Introduction
The molecular basis of beta thalassemia
Clinical aspects of beta thalassemia and related disorders
HbE disorders
Sickle Cell Disease: Introduction
Clinical and pathophysiological aspects of sickle cell anemia
Biology of pain and treatment of the sickle cell painful episode
HbSC disease and HbC
Sickle cell trait
Other sickle hemoglobinopathies
Other Clinically Important Disorders of Hemoglobin: Introduction
Unstable hemoglobins; hemoglobins with altered oxygen affinity; hemoglobin M; other variants of clinical and biological interest
Special Topics in Hemoglobinopathies: Introduction
Population genetics and global health burden
Genetic modulation of sickle cell disease and thalassemia
Developments in laboratory methods to detect hemoglobinopathies
New Approaches to the Treatment of Hemoglobinopathies and Thalassemia: Introduction
Transfusion and iron chelation
Induction of HbF
Novel approaches to treatment - (anitoxidants, statins, anti-inflammatory agents, K-Cl and Gardos inhibitors, other transport inhibitors, NO) Carlo Brugnara, Kirkwood A. Pritchard,
Stem cell transplantation Emanuele Angelucci and Mark Walters
Gene therapy