Griffin P Rodgers MDMACP - The Bethesda Handbook of Clinical Hematology, 4e

THE BETHESDA HANDBOOK

OF CLINICAL HEMATOLOGY

FOURTH EDITION

EDITORS

GRIFFIN P. RODGERS, MD, MACP

Chief
Molecular and Clinical Hematology Branch
National Heart, Lung, and Blood Institute

Director
National Institute of Diabetes and Digestive and Kidney Disease
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

Senior Acquisitions Editor: Ryan Shaw

Editorial Coordinator: Kerry McShane

Editorial Assistant: Amy Masgay

Marketing Manager: Rachel Mante Leung

Production Project Manager: David Saltzberg

Design Coordinator: Stephen Druding

Manufacturing Coordinator: Beth Welsh

Prepress Vendor: Newgen Knowledge Works Pvt. Ltd., Chennai, India

Fourth edition

Copyright 2019 Wolters Kluwer.

Copyright 2013, 2010 Lippincott Williams & Wilkins, a Wolters Kluwer business.

Copyright 2005 by Lippincott Williams & Wilkins.

All rights reserved. This book is protected by copyright. No part of this book may be reproduced or transmitted in any form or by any means, including as photocopies or scanned-in or other electronic copies, 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. To request permission, please contact Wolters Kluwer at Two Commerce Square, 2001 Market Street, Philadelphia, PA 19103, via email at (products and services).

9 8 7 6 5 4 3 2 1

Printed in China

9781496354037

2017057997

Library of Congress Cataloging-in-Publication Data

available upon request

This work is provided as is, and the publisher disclaims any and all warranties, express or implied, including any warranties as to accuracy, comprehensiveness, or currency of the content of this work.

This work is no substitute for individual patient assessment based upon health care professionals examination of each patient and consideration of, among other things, age, weight, gender, current or prior medical conditions, medication history, laboratory data, and other factors unique to the patient. The publisher does not provide medical advice or guidance and this work is merely a reference tool. Health care professionals, and not the publisher, are solely responsible for the use of this work including all medical judgments and for any resulting diagnosis and treatments.

Given continuous, rapid advances in medical science and health information, independent professional verification of medical diagnoses, indications, appropriate pharmaceutical selections and dosages, and treatment options should be made and health care professionals should consult a variety of sources. When prescribing medication, health care professionals are advised to consult the product information sheet (the manufacturers package insert) accompanying each drug to verify, among other things, conditions of use, warnings and side effects and identify any changes in dosage schedule or contraindications, particularly if the medication to be administered is new, infrequently used, or has a narrow therapeutic range. To the maximum extent permitted under applicable law, no responsibility is assumed by the publisher for any injury and/ or damage to persons or property, as a matter of products liability, negligence law or otherwise, or from any reference to or use by any person of this work.

LWW.com

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

Life is short, the art long

Hippocrates (c. 460 to 357 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 The Bethesda Handbook of Clinical Hematology 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. The Bethesda Handbook of Clinical Hematology 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 fourth edition of The Bethesda Handbook of Clinical Hematology, 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 The Bethesda Handbook of Clinical Hematology available as a handheld application. The Bethesda Handbook of Clinical Hematology 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 challenging from 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.

Griffin P. Rodgers, MD, MACP