Griffin P. Rodgers - The Bethesda Handbook of Clinical Hematology

THE BETHESDA
HAND BOOK

OF CLINICAL
HEMATOLOGY

THIRD EDITION

EDITORS

GRIFFIN P. RODGERS , MD, MACP

Chief, National Institute of Diabetes and Digestive and Kidney Diseases Chief, Molecular and Clinical Hematology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland

NEAL S. YOUNG , MD, MACP

Chief, Hematology Branch, National Heart, Lung, Blood Institute and National Institutes of Health Bethesda, Maryland

Senior Executive Editor: Jonathan W. Pine, Jr.

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2013 by Lippincott Williams & Wilkins, A Wolters Kluwer business

2010 by LIPPINCOTT WILLIAMS & WILKINS, a WOLTERS KLUWER business

2005, First Edition, Lippincott Williams & Wilkins

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All rights reserved. This book is protected by copyright. No part of this book may be reproduced in any form by any means, including photocopying, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above- mentioned copyright.

Printed in China

Library of Congress Cataloging-in-Publication Data

The Bethesda handbook of clinical hematology / editors, Griffin P. Rodgers, MD, MACP, director, National Institute of Diabetes, Digestive, and Kidney Disorders, chief, Molecular and Clinical Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health Bethesda, Maryland, Neal S. Young, MD, MACP, chief, Hematology Branch National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland. Third edition.

pages cm

Includes bibliographical references and index.

ISBN 978-1-4511-8270-5

1. BloodDiseasesHandbooks, manuals, etc. 2. HematologyHandbooks, manuals, etc. I. Rodgers, Griffin P., editor of compilation. II. Young, Neal S., editor of compilation. III. Title: Handbook of clinical hematology.

RC633.B49 2013

616.15dc23

2012049314

Care has been taken to confirm the accuracy of the information presented and to describe generally accepted practices. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. Application of the information in a particular situation remains the professional responsibility of the practitioner.

The authors, editors, and publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accordance with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new or infrequently employed drug.

Some drugs and medical devices presented in the publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings. It is the responsibility of the health care provider to ascertain the FDA status of each drug or device planned for use in their clinical practice.

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For our children, with love: Chris and Gregory Rodgers Andrea, Max, and Giorgio Young

PREFACE

Life is short, the art long

Hippocrates c. 460357 BC

The accessibility of blood and bone marrow has made hematology historically the engine of basic research in internal medicine. Hematology has thrived at the National Institutes of Health because of this close relationship with the research laboratory, and investigators from the various institutes in Bethesda have contributed to the knowledge of blood diseases from the study of individual patients with sometimes rare diseases and to the development of clinical protocols for the rigorous assessment of diagnostic criteria or treatments, both established and novel. Our hematology fellowship programs have fostered a scientific approach to hematology, not only to assess outcomes but also to advance the experimental basis of our understanding of blood diseases and the application of laboratory insights to their treatment in practice. The collegial relationships among local institutions and individuals in the greater Washington area who share training and patients have greatly furthered these efforts.

HANDBOOKS are intended to be highly accessible, both literally and figuratively, so as to be useful. Our HANDBOOK should be carried in the white coat pocket of the student, resident, and fellow on a hematology or oncology service and in the briefcase of the internist, hospitalist, family practitioner, and pediatrician whose practice includes patients with blood diseases. We have purposely combined authors who are recognized experts in their fields with senior fellows who have had current experience of learning hematology and daily care of hematology patients, and encouraged a thoughtful approach to the presentation of the core knowledge, using tables, algorithms, meaningful figures, and bulleted text structures[CE3]. The HANDBOOK is organized according to disease categories and hematological problems of importance to the consulting and treating hematologist, and additional chapters are provided to acquaint the reader with familiar and new laboratory methodologies that underlie modern clinical approaches to diagnosis and treatment.

For the opportunity to publish a third edition of the BHOCH, we thank our readers, who have supported our efforts with both kind comments and constructive criticisms and, very importantly, concrete purchases of the book, more remarkable in an age of Internet-based medical information. Indeed, the plan is to have this BHOCH available as a handheld application. The HANDBOOK remains focused on providing the practitioner at every level of training practical, authoritative, and current guidance to the diagnosis and treatment of blood diseases and to consultative problems in hematology. Many aspects of hematology, as the field advances, have entered the domain of internal medicine but remain complex and challengingfrom new anticoagulants to the mundane management of once fatal diseases like chronic myeloid leukemia and aplastic anemia.

All chapters have been revised and updated. We look forward to your responses to our authors efforts.