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This world-class group of editors, with roots in academic medicine, basic research, industry, and government, have used their breadth of perspective and experience to organize a well-balanced and timely textbook. They have superbly organized the work of 49 contributors, leaders in their fields, to cover a range of diseases for which there is extensive molecular knowledge.
This expanded and updated third edition is timely, since the second edition was published seven years ago. The book is divided into eight parts that cover hematopoiesis, red cells, lymphopoiesis, myelopoiesis and white cells, hemostasis, molecular oncology, viruses, and gene therapy. The second edition contained a section on basic concepts, including the methods of molecular genetics and the mechanisms of eukaryotic gene regulation, which has been omitted from the third edition. This omission reflects a rational editorial decision, since discussions of these topics are widely available in other specialty textbooks, and the omission of this section provided space needed for the coverage of new developments in molecular hematology. Chapters on the molecular biology of enzymes involved in erythrocyte metabolism and on the molecular basis of white-cell motility, which were in the second edition, have been replaced in the current edition by seven new chapters, resulting in a greater breadth of coverage. The new chapters discuss areas in which there has been an exponential increase in molecular information since the previous edition: stem-cell biology, hematopoietic growth factors and receptors, hematopoietic transcription factors, signal transduction in the regulation of hematopoiesis, integrins in hematology, paroxysmal nocturnal hemoglobinuria, and gene therapy for blood diseases. Each chapter is extensively referenced, with citations as recent as 1999.
This book is not a classic textbook of hematology. The description of many diseases for which we lack detailed molecular knowledge is left to the comprehensive hematology textbooks. Differential diagnoses, clinical decision making, and treatment approaches are not discussed in a manner that parallels the in-depth presentation of scientific information. For example, the chapter on gene rearrangements in lymphoid cells covers many topics, including immune receptors as markers of clonal proliferation and participants in the generation of chromosomal aberrations. Although this chapter also provides a nice overview of the use of polymerase-chain-reaction analysis to detect gene rearrangements and minimal residual disease in patients with hematologic neoplasms, there is no in-depth analysis of its role in treatment or clinical management. Similarly, although this chapter covers what is known about the mechanism of V(D)J recombination and the structure and function of immunoglobulins, there is no discussion of the use of immunoglobulins in the treatment of immunocompromised patients.
These intentional omissions help keep the book focused on the science underlying mechanisms of disease rather than on clinical hematology. When appropriate, however, clinical correlates are often cited to bring a clinical perspective to the scientific information presented. For example, there is a discussion of severe thrombophilia as a multigenetic disease in the chapter on the protein C anticoagulant system. In addition, a scientifically comprehensive, well-written chapter on the molecular and biologic properties of the human immunodeficiency virus is followed by thoughtful sections on clinical perspectives on the life cycle of the virus and on the prospects for developing a vaccine against human immunodeficiency virus type 1. Nevertheless, the reader should not expect this book to serve as a resource for evidence-based clinical hematology or for in-depth guidelines on the treatment and management of hematologic disorders. This work should therefore be considered a supplement to a basic hematology textbook. If the book is viewed in this light, readers will not be disappointed.
Rapid developments and continuous progress in the molecular biology and biochemistry of blood diseases make it increasingly difficult to maintain an up-to-date knowledge base in all areas of hematology. The new edition of this textbook is a useful resource for learning about the molecular aspects of hematologic disorders. The practical aspects of publishing make it difficult, if not impossible, to produce a scientific textbook that is truly up to date -- a problem that is evident in this book, with references that are already one or two years out of date. To satisfy the needs of the readership for which the editors claim this book has been written, future editions may require linkage to an internet-based electronic supplement, so that timely updates and references can be provided. Nonetheless, this third edition is a wonderful book.
David A. Roth, M.D.
Copyright © 2001 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS.