The Thalassaemia Syndromes

by Weatherall, David J.; Clegg, J. B.

• ISBN13: 9780470696705
• ISBN10: 0470696702
• Edition: 4th
• Format: eBook
• Copyright: 2008-04-16
• Publisher: Wiley-Blackwell

Summary

In the new edition of this successful and authoritative book, the thalassaemias are reviewed in detail with respect to their clinical features, cellular pathology, molecular genetics, prevention and treatment. It is aimed at specialists in haematology in the laboratory or clinical setting, particularly in areas where thalassaemia is common either in the native population or in immigrant communities.

The fourth edition has been both updated and re-organized. Three new chapters have been added on the link between alpha-thalassaemia and mental retardation, on avoidance and population control and on global epidemiology. Considerable emphasis is placed on molecular pathology reflecting the huge burst of information to have come out of this field in the last few years.

Table of Contents

Authors and Contributors.

Preface to the Fourth Edition.

Preface to the First Edition.

Acknowledgements.

Part 1 Historical Background.

1. Historical Perspectives: The Many and Diverse Routes to our Current Understanding of the Thalassaemia Syndromes.

Part 2 The Biology of The Thalassaemias.

2. Human Haemoglobin.

3. Thalassaemia: Classification and Relationship to Other Inherited Diseases of Haemoglobin.

4. The Molecular Pathology of the Thalassaemias.

5. Pathophysiology of the Thalassaemias.

6. The World Distribution and Population Genetics of Thalassaemia.

Part 3 Clinical Features of the Thalassaemias .

7. The beta Thalassaemias.

8. The delta beta and Related Thalassaemias.

9. The beta and delta beta Thalassaemia in Association with Structural Haemoglobin Variants.

10. Hereditary Persistence of Fetal Haemoglobin.

11. The alpha Thalassaemias and Their Interactions with Structural Haemoglobin Variants.

12. Thalassaemia withmental retardation or Myelodysplasia.

13. Thalassaemia Intermedia.

Part 4 Diagnosis and management of thalassaemia. .

14. Avoidance and Population Control.

15. Management and prognosis.

16. Laboratory Diagnosis of the Thalassaemias.

Part 5 The Future.

References.

Appendix.

Index.