Elsevier 30 Corporate Drive, Suite 400, Burlington, MA 01803, USA 32 Jamestown Road, London NW1 7BY, UK 525 B Street, Suite 1900, San Diego, California 92101-4495, USA 360 Park Avenue South, New York, NY 10010-1710, USA First edition 2009 Copyright © 2009, Elsevier Inc. Apart from Chapters 115, 116, 117, 118, 122, 123, 124, 125, 126, 127, 128 which are in the public domain. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system or transmitted in any form or by any means electronic, mechanical, photocopying, recording or otherwise without the prior written permission of the publisher Permissions may be sought directly from Elsevier’s Science & Technology Rights Department in Oxford, UK: phone: (44) (0) 1865 843830, fax: (44) (0) 1865 853333, E-mail: [email protected]. 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It is the responsibility of the treating physician, relying on experience and knowledge of the patient, to determine dosages and the best treatment for each individual patient. Neither the publisher nor the authors assume any liability for any injury and/or damage to persons or property arising from this publication. Library of Congress Cataloging in Publication Data A catalog record for this book is available from the Library of Congress British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library ISBN: 978-0-12-374432-6 For all information on all Elsevier publications visit our Web site at www.elsevierdirect.com Typeset by Macmillan Publishing Solutions (www.macmillansolutions.com) Printed and bound in United States of America About the Editors Christopher D. Hillyer, MD, is the Emory Distinguished Service Professor of Pathology, and atenured professor in the Department of Pathology and Laboratory Medicine, Emory University School of Medicine. In these capacities, he serves as direc- tor of the Emory Center for Transfusion and Cellular Therapies with responsibility for all aspects of clinical and academic transfusion medicine at Emory’s seven principle hospitals. Dr. Hillyer is chief editor of three textbooks in transfusion medicine, author of over 120 articles pertaining to transfusion, human immunodeficiency virus (HIV), cytokines, and herpes viruses, most notably Cytomegalovirus (CMV), as well as more than 25 book chapters. Nationally recognized as an expert in hematology blood bank- ing, and transfusion medicine, Dr. Hillyer also is a past-president, board of directors of AABB (formerly known as the American Association of Blood Banks) and a former trustee of the National Blood Foundation (NBF). Dr. Hillyer has been awarded many million dollars in research funding from the National Institutes of Health (NIH), the Centers for Disease Control and Prevention (CDC), NBF and other agencies. He is an associate editor of the journal Transfusion and serves on several other editorial boards including the journal Blood. Dr. Hillyer was recently recognized as a Healthcare Hero for his work in Africa and is a recipient of the Tiffany Award from the American Red Cross where he also serves as a medical director. Dr. Hillyer is board certified in four specialty areas including transfusion medicine, hematology, medical oncology and internal medicine. He received his BS from Trinity College (1980), and his MD from the University of Rochester School of Medicine (1984), with postgraduate training and fellowships in hematology-oncology, transfusion medicine and bone marrow transplantation at Tufts-New England Medical Center in Boston. Beth H. Shaz, MD, is an associate professor in the Department of Pathology and Laboratory Medicine, Emory University School of Medicine, and serves as director of the blood bank at Grady Memorial Hospital and co-director of clinical research at the Center for Transfusion and Cellular Therapy. Prior to moving to Emory she was the assistant medical director of the Transfusion Services and medical director of the Transfusion and Apheresis Unit at Beth Israel Deaconess Medical Center. She has writ- ten many articles and book chapters pertaining to transfusion, cellular therapy, and therapeutic apheresis. Nationally, she is chair of the Clinical Applications Committee of the American Society for Apheresis, which has recently published new and evi- dence-based guidelines for the use of therapeutic apheresis. In addition, she is the chair of the Transfusion Services Accreditation Program Unit and the Blood Banking/ Transfusion Medicine Fellowship Program Directors Subsection of the AABB. Dr. Shaz is board certified in transfusion medicine, clinical and anatomic pathol- ogy. She received her BS from Cornell University and her MD from the University of Michigan School of Medicine. Her postgraduate training includes a general surgery internship at Georgetown University, and residency in clinical and anatomic pathology xv xvi AbouttheEditors and fellowship in transfusion medicine at Beth Israel Deaconess Medical Center, a major teaching hospital of Harvard Medical School in Boston. James C. Zimring, MD, PhD, is an tenured associate professor in the Department of Pathology and Laboratory Medicine, Emory University School of Medicine, and serves as the assistant director of the special hemostasis laboratory at Emory University Hospital. Dr. Zimring oversees the laboratory diagnosis of patients with disorders in thrombosis and hemostasis. In addition, Dr. Zimring has an active NIH funded research program studying the immunology of transfusion and bone mar- row transplantation. Dr. Zimring is a recent recipient of the David B. Pall award for advances in transfusion medicine, and frequently presents original research in the field of transfusion at national and international meetings. He is on the editorial board of the journal Transfusion and serves as a reviewer for additional journals in the fields of transfusion and coagulation. Dr. Zimring has authored several book chapters in the fields of transfusion, coagulation and molecular pathology. He is board certified in Clinical Pathology and received a BS in Chemistry, PhD in Immunology, and an MD all from Emory University. Thomas C. Abshire, MD, is a professor in the Department of Pediatrics Emory University School of Medicine, and the director of the Comprehensive Hemostasis Program, Emory University and Children’s Healthcare of Atlanta. He also serves as the Pediatric Hematology/Oncology Fellowship Program Director at Emory and Children’s. Prior to joining the faculty at Emory University, Dr. Abshire completed a career in the United States Air Force with an emphasis as a clinician-educator, where he was the recipient of several medical student and housestaff teaching awards. Dr. Abshire’s research interest is in evaluation of mild bleeding disorders and therapeutic intervention in hemophilia, von Willebrand disease and thrombosis. He has published over 60 peer-reviewed manuscripts, review articles or editorials and is a frequent invited speaker at national and international meetings. He also serves on the Steering Committee of the International Immune Tolerance Study and recently was a member on the CDC Uniform Data Collection Committee. He has received research funding from the NIH, CDC, and several non profit organizations and pharmaceutical com- panies. Dr. Abshire is a past-president of the Hemophilia and Thrombosis Research Society. Dr. Abshire is a distinguished graduate of the US Air Force Academy. He attended medical school at Tulane University, fulfilled his pediatric residency training at David Grant USAF Medical Center and completed pediatric hematology/oncology fellowship training at the University of Colorado Health Science Center. Contributors THOMAS C. ABSHIRE, MD Professor, Department of Pediatrics, Emory University School of Medicine Director’s Chair in Hemostasis, Hemophilia of Georgia, Inc. Director, Comprehensive Hemostasis Program, Emory University and Children’s Healthcare of Atlanta, Atlanta, Georgia, USA CAROLYN M. BENNETT, MD Assistant Professor, Department of Pediatrics, Emory University School of Medicine Attending Physician, Aflac Cancer Center and Blood Disoders Service, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA MICHAEL A. BRIONES, DO Assistant Professor, Department of Pediatrics, Emory University School of Medicine Attending Physician, Aflac Cancer Center and Blood Disoders Service, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA MARY D. DARROW, MD Resident Physician, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA AMY L. DUNN, MD Assistant Professor, Department of Pediatrics, Emory University School of Medicine Attending Physician, Aflac Cancer Center and Blood Disoders Service, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA JIMMIE L. EVANS, MT (ASCP) Manager, Transfusion Medicine Laboratory Services, Emory University Hospital, Atlanta, Georgia, USA LAWRENCE B. FIALKOW, DO Fellow, Center for Transfusion Medicine and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA COURTNEY E. GREENE, MD Resident Physician, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA ALFRED J. GRINDON, MD Professor Emeritus, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA xvii xviii Contributors ELEANOR S. HAMILTON, MT (ASCP) Medical Technologist, Cellular Therapies Laboratory, Emory University Hospital, Atlanta, Georgia, USA JEANNE E. HENDRICKSON, MD Assistant Professor, Department of Pediatrics, Emory University School of Medicine Assistant Director, Blood Banks and Transfusion Services, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA CHRISTOPHER D. HILLYER, MD Distinguished Service Professor of Pathology Professor, Department of Pathology and Laboratory Medicine, Emory University School of Medicine Professor, Department of Hematology and Medical Oncology, Emory University School of Medicine Director, Center for Transfusion and Cellular Therapies, Atlanta, Georgia, USA KRISTA L. HILLYER, MD Assistant Professor, Department of Pathology and Laboratory Medicine, Emory University School of Medicine Chief Medical Officer, Southest Division, American Red Cross Blood Services, Douglasville, Georgia, USA SHAWN M. JOBE, MD, PhD Assistant Professor, Department of Pediatrics, Emory University School of Medicine Attending Physician, Aflac Cancer Center and Blood Disoders Service, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA CASSSANDRA D. JOSEPHSON, MD Associate Professor, Department of Pathology and Laboratory Medicine, Emory University School of Medicine Associate Director, Blood Banks and Transfusion Services, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA CHRISTINE L. KEMPTON, MD Assistant Professor, Department of Pediatrics and Department of Hematology and Medical Oncology, Emory University School of Medicine Assistant Professor, Attending Physician, Aflac Cancer Center and Blood Disoders Service, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA SHANNON L. MEEKS, MD Instructor, Department of Pediatrics, Emory University School of Medicine Attending Physician, Aflac Cancer Center and Blood Disoders Service, Children’s Healthcare of Atlanta, Atlanta, Georgia, USA CONNIE H. MILLER, PhD Director, Clinical Hemostasis Laboratory, Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia, USA Preface The editors present this inaugural edition of Transfusion Medicine and Hemostasis – Clinical and Laboratory Aspects with great excitement and anticipation. As the title indicates, we view, and have done so for some time, that both transfusion medicine and hemostasis, particularly laboratory methods and diagnostic tests employed in routine and specialized coagulation laboratories, are linked. More specifically, coagu- lation tests, and indeed other laboratory tests and results, are often used to guide and monitor transfusion, blood component and coagulation factor therapy. Also, transfu- sion strategies are used to treat patients with disorders of hemostasis which have been previously diagnosed using specialized and vital hemostasis and coagulation laboratory tests and methods. In addition, pathology, pediatric and internal medicine residents, and transfusion medicine and hematology fellows, often receive training (to varying extents) in blood banking and transfusion medicine, and in disorders and diseases of congenital and acquired coagulation defects, though a combined and appropriately focused book does not appear to exist. Moreover, books that focus on critical and technical elements of laboratory testing methodologies, in the context of transfusion and hemostasis, are few – if any. Indeed, in our institution both transfusion medicine and the coagulation/ hemostasis laboratories are housed in the same clinical and administrative place – the Emory Center for Transfusion and Cellular Therapies – and joint consultation services exist. It is thus that we envisioned a manual-style book combining transfusion medicine and hemostasis/coagulation medicine. Indeed, the book before you is divided into these two main parts, as the title obviously suggests. Not surprisingly, others have noted the interdigitation of these two fields – such as those who conceived the Transfusion Medicine/Hemostasis Clinical Trials Network, now funded by the National Institutes of Health. Also, board certification in blood banking and transfusion medicine is currently accomplished via the American Board of Pathology, and the examination has a significant emphasis on clinical hemostasis and laboratory aspects of coagulation as well as blood banking and transfusion medicine. Interestingly, board eligibility in transfusion medicine, following an accredited transfusion medicine fellow- ship, can be achieved directly via internal medicine, pediatrics and anesthesia residencies, in addition to pathology and adult and pediatric hematology. Thus, it is our intention that this offering be of value to trainees in many specialties, as well as fellows in transfusion medicine and hematology. Moreover, as transfusion is the most common therapeutic modality in use in the US, it is anticipated that this book will provide valuable information for practitioners in a variety of fields, including anesthesiology, surgery and obstetrics. The format of this book is intentional, with shorter, concise chapters being the norm. In each chapter, the intent is to have clear paragraphs, bullet points, and tables and figures, with a hierarchy of headings so that presentation of key points will be most efficient. Where possible and appropriate, a common hierarchical format has xxi xxii Preface been employed. Instead of an exhaustive list of references, the editors have chosen to offer a small number of key articles from which the reader may gain additional information. Even without the usual citation-based technical writing style typically employed in books, we have tried to incorporate evidence-based and data-driven prose, and have referred to specific authors, studies, and publications in a way that will allow the reader to find a specific reference even when it is not cited at the end of a chapter. We fervently hope this style will be efficient, informative and enjoyable. As stated above, the book is divided into two main parts – transfusion medicine and hemostasis. That on transfusion medicine is further subdivided into blood banking (that is, blood collection, processing, testing and storage) and transfusion medicine (that is, components for transfusion, pretransfusion immunohematology testing, product modi- fications, approaches to transfusion therapy in specific clinical settings, and transfusion reactions and complications). Also included are apheresis, cellular therapy, and an intro- duction to tissue banking in the hospital setting. These chapters and sub-parts follow introductory chapters with common elements defining the field of blood banking and transfusion medicine, a brief history, and quality and regulatory principles. We have also included chapters focused on the role of the physician in both the blood collection facil- ity and the transfusion service, as well as descriptions of careers in the field. In the second main part of the book, dedicated to hemostasis, the chapters are further divided into sub-parts – clinical coagulation, laboratory-based coagulation testing, and coagulation factor products. While these three sub-parts could have been compressed into a disease-based chapter system, we felt that focus on the clinical issues, the labora- tory and testing methods and details to diagnose and guide and monitor therapy, and finally the products available to treat the conditions, would be helpful and ease assimila- tion of the material. We hope you will agree. Finally, in many places throughout the book, we have sections or paragraphs dedi- cated to international considerations. These are not exhaustive and were not meant to be, especially as regulatory requirements and practice variations vary widely through- out the developed and the developing, world. Nonetheless, the editors felt it was important to broaden the horizon of this book to have somewhat of a “world view.” Again, we hope you will agree. In closing, we are deeply dedicated to the fields of transfusion medicine and hemosta- sis, the ambition of striving towards optimal diagnosis, laboratory testing and therapies for patients worldwide, and the education of trainees, practitioners and future leaders in these inter-related disciplines. We hope this book will fill a void, achieve a new vision, and perhaps be seen as unique and inspired in its presentation. We ask sincerely for feed- back, general or specific criticism, and suggestions as to how to improve this book, and thank you for the confidence and trust you have placed in the authors and editors. Christopher D. Hillyer, MD Beth H. Shaz, MD Thomas C. Abshire, MD James C. Zimring, MD, PhD Acknowledgments We, the editors, would like to acknowledge the outstanding technical and profes- sional support of Sue Rollins, and the expertise and guidance of Megan Wickline and Mara Conner and other team members at Elsevier. Each of these individuals played an instrumental role in the creation of this textbook, and we sincerely thank them. We would like to thank our friends and families for their unconditional love and sup- port, without which this project could not have come to fruition. We thank especially Krista, Whitney, Peter, Margot, Jackson and James Hillyer; David, Samara, Jacob and Andrew Shaz; Kim Jallow and Alexandra Zimring; and Diane, Jonathan, Jennifer, Erin and Matthew Abshire. xxiii P A R T I Blood Banking and Transfusion medicine
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