Disorders of Hemoglobin
Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management
Editors: Martin H Steinberg, Bernard G Forget, Douglas R Higgs & Ronald L Nagel
Cambridge University Press, UK, 2001; 1268 pages.
This large multiauthor work devoted to all aspects of hemoglobin disorders has been edited by an internationally renowned group of researchers who have selected a team of 53 contributors, each of which has produced clear, authoritative chapters. The editors have done a good job and have managed to give the book a fairly uniform feel, something which is often lacking in edited texts.
The historical perspective provided at the beginning of the book is very useful and highly readable. Junior staff in hematology often have limited knowledge of the discoveries in the 1950s and 1960s that led to the understanding of the 3-dimensional structure and function of the hemoglobin molecule. Rereading the details of this pioneering research is fascinating. The chapter devoted to the molecular anatomy and physiology of hemoglobin was written by Max Perutz, the Nobel prize-winner who contributed so much to this area of medicine, so it is especially apt to have him amongst the contributors.
Following the physiology, embryology and evolutionary aspects, the book progresses through fairly predictable sections dealing with B thalassemia, a thalassemia and sickle cell disease. The editors have ensured that recent trial data are included and full details regarding the laboratory diagnosis and clinical management are included. I found the chapter on the acute painful episode in sickle cells disease particularly good.
A later section is devoted entirely to laboratory diagnostic aspects, including prenatal diagnosis, and provides very useful details of DNA-based techniques for diagnostic use. Dr Old has provided full details of primers required for PCR amplification for diagnosis of thalassemia. This section will be extremely useful for those wishing to set up laboratories for the diagnosis of hemoglobin disorders.
Later sections deal with transfusion therapy in hemoglobin disorders, transplantation, gene therapy and experimental treatments. This is followed by a chapter dealing with high and low affinity hemoglobins, which might have been better placed earlier in the book.
Overall, I enjoyed browsing through this text. There is truly something for everyone. It will doubtless become the hemoglobinopathy reference 'bible'. It will be invaluable to clinicians who care for patients with hemoglobin disorders, as well as laboratory researchers who are involved in hemoglobinopathy research.
My only minor quibbles are that the text is slightly difficult to read (for me at least) because the letter spacing is a little loose. The artwork was patchy and largely black and white; some figures were out of focus and some should have been redrawn. There is a useful color section in the center of the book, which is necessary for the display of detailed illustrations, photomicrographs, and many of the other illustrations that do not reproduce well in black and white.
Editing a text of this size represents a huge amount of work for the editors and they must be congratulated in producing such a high quality and immensely useful and readable book which will aid the diagnosis, management and overall understanding of these common disorders.
Reviewed for Bloodline by:
Drew Provan, MD
Department of Haematology
The Royal London Hospital
London, England
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