Summary :
Preface The rate of growth in our understanding of diseases of blood cells and coagulation proteins provides a challenge for the editors of a comprehensive textbook of hematology. The completion of the genome project and the acquisition of knowledge in the fields of genomics and proteomics, as applied to hematologic disorders, have accelerated the understanding of the pathogenesis of the diseases of our interest. The rate at which basic knowledge in molecular and cell biology and molecular immunology has been translated into improved diagnostic and therapeutic methods is equally as impressive. The vision of specific molecular targets for therapy has become a reality for some diseases and more is sure to come. In response to the increased number of facts amassed in hematology and the complexity of integrating these facts for the reader, the Editorial Board of Williams Hematology has added two new members, Josef T. Prchal and Kenneth Kaushansky. Drs. Prchal and Kaushansky bring special expertise in genetics, red cell and platelet disorders, hematopoiesis, and cell signaling, as well as a broad knowledge of clinical hematology, to our work. It is that combination of breadth and depth that permits them to have a significant impact on the quality of this textbook. In this transition, Barry Coller who participated in the 4th and 5th editions decided to retire from the Board as he took on new responsibilities at Rockefeller University. Three past contributors died during the preparation of the book, Alan Erslev, Bernard Babior, and David Golde. Alan Erslev was a member of the editorial board for the first four editions of the book and continued as a contributor to the 5th and 6th editions. He was a person of intellect and integrity, which made his contributions especially meaningful. Each of these accomplished scholars was a friend, a colleague, and a valued participant. We share with their families and with their other friends and colleagues a deep sense of loss. This edition of the book has undergone a significant reorganization. The section on Therapeutic Principles has been expanded to include new chapters: Principles of Antithrombotic Therapy, Immune Cell Therapy, and Vaccine Therapy, along with revised versions of Principles of Chemotherapy, Hematopoietic Stem Cell Transplantation, Hemapheresis, Gene Therapy, and Management of Infections in the Compromised Host. Other new chapters, “The Innate Immune System,” “The Adaptive Immune System and Dendritic Cells,” “Antibody-Mediated Coagulation Factor Deficiencies,” “Antibody-Mediated Thrombotic Disorders,” and “Hematologic Considerations in Pregnancy,” have been added. Each chapter has been extensively revised or rewritten to provide the most current information available. Several areas in which there were separate chapters on basic science aspects of a topic and on relevant clinical disorders have been combined so that both basic and clinical material are found in one chapter. We believe that this organization makes it easier for readers to access the full range of information they may want, and it reduces duplication of text and references. These chapters include (a) the reorganization of material in separate chapters related to oral anticoagulant, heparin, and fibrinolytic therapy, and antiplatelet agents into a new chapter, “Principles of Antithrombotic Therapy,” (b) the combination of the metabolism of folate and cobalamin and megaloblastic anemias into a single chapter, (c) the consolidation of iron metabolism, iron transport, iron deficiency, and iron storage diseases, (d) coupling energy metabolism of red cells with hemolytic anemia resulting from glucose-6-phosphate dehydrogenase and other enzyme deficiencies, (e) tying together the structure and biochemistry of the red cell membrane with hemolysis resulting from hereditary membrane disorders, (f) integrating hemoglobin structure and function with the inherited hemoglobin disorders, (g) combining traumatic, microangiopathic, march, and sports anemias into one unit, (h) condensing warm, cold, and drug-induced antibody-mediated hemolytic anemia into one chapter on immune hemolytic disorders, and (i) uniting the chapter on functions of neutrophils with qualitative abnormalities of the neutrophil. Each chapter has a primary editor who interacts with the author and reviews the chapter, making suggestions ranging from grammar to organization to content. Thereafter, each of the other editors is sent the chapter and asked to comment. Suggestions are incorporated before the chapter is submitted to the publisher. This ensures that the author gets several reviews of the chapter, the assigned primary and secondary editor and any additional editors that may comment. The goal is to have each chapter (a) be informative in the fewest words possible, (b) avoid unnecessary duplication among chapters, (c) provide balance between basic science and clinical information, (d) contain cross-referencing to other relevant chapters, (e) include current and carefully selected references, (f) encourage the use of review articles among the references, and (g) display informative tables and figures. The production of this book required the timely cooperation of 150 contributors. We are grateful for their work in providing this comprehensive and up-to-date text. Despite the growth of both basic and clinical knowledge and the passion that each of our contributors brings to the topic of their chapter, we have been able to maintain the text in a single volume, through scrupulous attention to chapter length. We also include twenty-five color plates that refer to blood, marrow, and lymph node pathology, cytogenetics, gene expression profiles, and patient clinical findings that are best illustrated in color. We thank Jean Shafer, B.S., M.A., of the University of Rochester Medical Center for providing the blood and marrow cell color images on Plates I through XXI-9; Bartel Barlogie and colleagues for providing the images of myeloma cells and gene expression profiles of normal plasma cells and myeloma cells for Plate XXI-10 through 13; Randy D. Gascogne, M.D., of the British Columbia Cancer Agency, for the images of lymph node pathology and gene expression profiling of lymphoma in Plate XXII-3 through 40; Michelle M. LeBeau, Ph.D., and Lucy A. Godley, M.D., Ph.D., of the University of Chicago Medical Center, for Plate XXIV on cytogenetics; Marissa Braff, M.D., Magdalene Dohil, M.D., Terrance O'Grady, M.D., Stephanie Sturgill, M.D., and William Sturgill, M.D., of the University of California, San Diego, for images on Plate XXV-6, 9, 13, 15, 25, 36, and 37, respectively; Virgil Fairbanks, M.D., of the Mayo Clinic, for image XXV-43; Karl Blume, M.D., of Stanford University for images on Plate XXV-44 and 45; Harold Roberts for image on Plate XXV-46; and Paul Schneiderman, M.D., Columbia University, for the other images of cutaneous abnormalities used in Plate XXV. This edition represents the first that will be also available in electronic form on the McGraw-Hill Book Company website, AccessMedicine.com, which contains a variety of major textbooks and other sources. Periodic updates will enhance the timeliness of information available on this site. An extensive atlas of blood, marrow, lymph node, cytogenetic, and flow cytometric images relevant to hematologic diagnosis is planned for the site as well.

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