Clinical Haematology Third Edition Clinical Haematology Third edition EDITED BY Drew Provan Senior Lecturer in Haematology Barts and The London School of Medicine London, UK © 2007 by Blackwell Publishing Ltd BMJ Books is an imprint of the BMJ Publishing Group Limited, used under licence Blackwell Publishing, Inc., 350 Main Street, Malden, Massachusetts 02148-5020, USA Blackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ, UK Blackwell Publishing Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia The right of the Author to be identifi ed as the Author of this Work has been asserted in accordance with the Copyright, Designs and Patents Act 1988. 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, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. First published 1998 Second edition 2003 Third edition 2007 1 2007 Library of Congress Cataloging-in-Publication Data ABC of clinical haematology / edited by Drew Provan. -- 3rd ed. p. ; cm. Includes bibliographical references and index. ISBN 978-1-4051-5353-9 1. Hematology--Outlines, syllabi, etc. I. Provan, Andrew. [DNLM: 1. Hematologic Diseases--diagnosis. 2. Hematologic Diseases-- physiopathology. 3. Hematologic Diseases--therapy. WH 120 A134 2007] RC633.A23 2007 616.1’5--dc22 2006035608 ISBN: 978-1-4051-5353-9 A catalogue record for this title is available from the British Library Cover image of human red blood cells in a vein is courtesy of iStockphoto.com Set in 9.25/12 pt Minion by Sparks, Oxford – www.sparks.co.uk Printed and bound in Singapore by Markono Print Media, Pte Ltd Associate Editor: Vicki Donald Editorial Assistant: Victoria Pittman Production Controller: Rachel Edwards For further information on Blackwell Publishing, visit our website: www.blackwellpublishing.com The publisher’s policy is to use permanent paper from mills that operate a sustainable forestry policy, and which has been manufactured from pulp processed using acid-free and elementary chlorine-free practices. Furthermore, the publisher ensures that the text paper and cover board used have met acceptable environmental accreditation standards. Blackwell Publishing makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check that any product mentioned in this publication is used in accordance with the prescribing information prepared by the manufacturers. The author and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this book. Contents Contributors, vii Preface to Third Edition, ix 1 Iron Defi ciency Anaemia, 1 Drew Provan 2 Macrocytic Anaemias, 6 A Victor Hoffbrand, Drew Provan 3 The Hereditary Anaemias, 11 David J Weatherall 4 Polycythaemia, Essential Thrombocythaemia and Myelofi brosis, 17 George S Vassiliou, Anthony R Green 5 Chronic Myeloid Leukaemia, 22 John M Goldman 6 The Acute Leukaemias, 27 Mark Cook, Charles Craddock 7 Platelet Disorders, 33 Marie A Scully, Samuel J Machin, R J Leisner 8 The Myelodysplastic Syndromes, 40 Paul A Cahalin, John A Liu Yin 9 Multiple Myeloma and Related Conditions, 45 Charles R J Singer 10 Bleeding Disorders, Thrombosis and Anticoagulation, 52 David M Keeling 11 Lymphoproliferative Disorders Including Chronic Lymphocytic Leukaemia, 57 Bronwen Shaw, Simon O’Connor, Andrew P Haynes 12 Stem Cell Transplantation, 67 Fiona Clark, Charles Craddock 13 Haematological Disorders at the Extremes of Life, 72 Carolina Lahoz, Tyrell G J R Evans, Adrian C Newland 14 Haematological Emergencies, 78 Jim Murray, Belinda Austen, Drew Provan 15 The Future of Haematology: the Impact of Molecular Biology and Gene Therapy, 85 Bella R Patel, Adele K Fielding Index, 95 v Contributors Belinda Austen David M Keeling Specialist Registrar in Haematology, University Hospital Birmingham NHS Consultant Haematologist, The Churchill Hospital, Oxford, UK Foundation Trust, Birmingham, UK Carolina Lahoz Paul A Cahalin Specialist Registrar, Barts and The London, Queen Mary’s School of Medicine Specialist Registrar in Haematology, Manchester Royal Infi rmary, Manches- and Dentistry, London, UK ter, UK R J Leisner Fiona Clark Consultant Haematologist, Great Ormond Street Hospital for Children NHS Consultant Haemato-Oncologist, Queen Elizabeth Hospital, Birmingham, Trust and University College London Hospitals NHS Trust, London, UK UK Samuel J Machin Mark Cook Professor of Haematology, Haemostasis Research Unit, University College Consultant Haemato-Oncologist, Queen Elizabeth Hospital, Birmingham, UK London, London, UK Charles Craddock Jim Murray Professor of Haemato-Oncology, Queen Elizabeth Hospital, Birmingham, UK Consultant Haematologist and Honorary Senior Lecturer, University Hospi- tal Birmingham NHS Foundation Trust, Birmingham, UK Tyrell G J R Evans Adrian C Newland Senior Lecturer, King’s College School of Medicine and Dentistry, London, UK Professor of Haematology, Barts and The London, Queen Mary’s School of Medicine and Dentistry, London, UK Adele K Fielding Simon O’Connor Senior Lecturer/Honorary Consultant in Haematology, Royal Free and University College Medical School, London, UK Consultant Haematopathologist, Nottingham University Hospitals Trust, Nottingham, UK John M Goldman Bella R Patel Fogarty Scholar, Haematology Branch, National Institutes of Health, Bethesda, MD, USA Clinical Research Fellow, Royal Free and University College Medical School, London, UK Anthony R Green Drew Provan Professor of Haemato-Oncology, Cambridge Institute for Medical Research, Cambridge, UK Senior Lecturer in Haematology, Barts and The London, Queen Mary’s School of Medicine and Dentistry, London, UK Andrew P Haynes Marie A Scully Senior Lecturer in Haematology, Nottingham University Hospitals Trust, Nottingham, UK Lecturer in Haematology, Haemostasis Research Unit, University College London, London, UK A Victor Hoffbrand Bronwen Shaw Emeritus Professor of Haematology, Royal Free and University College Medi- Specialist Registrar in Haematology, Nottingham University Hospitals Trust, cal School, London, UK Nottingham, UK vii viii Contributors Charles R J Singer Sir David J Weatherall Consultant Haematologist, Royal United Hospital, Bath, UK Regius Professor of Medicine Emeritus, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK George S Vassiliou John A Liu Yin Specialist Registrar in Haematology, Ipswich Hospital, Ipswich, UK Professor in Haematology, Manchester Royal Infi rmary, Manchester, UK Preface to Third Edition In the three years since the second edition of the ABC of Clinical elodysplasia, leukaemias and transplantation, lymphomas, as well Haematology, there have been further advances in our understand- as bleeding disorders. Other chapters have been extensively over- ing and therapies for many haematological diseases. For some disor- hauled and new co-authors included to ensure that the content is ders new classifi cation systems have been devised. For many disor- fresh and up to date. The book should be of value to a wide variety ders molecular techniques have provided major diagnostic tools and of readers including medical students, nurses, family doctors, and greater insight into the pathogenesis of the diseases themselves. other health professionals involved in the care of patients with hae- However, despite the complexity of modern clinical haematology, matological disorders. the aim of the ABC of Clinical Haematology is to provide an over- Of course, the quality of the content and the writing are key fac- view of each disease area, with each chapter written by recognized tors in the success of a book such as this and I am indebted to my experts in their respective areas. The structure of the book remains haematology colleagues who have contributed high quality chapters true to the original ABC ethos with succinct text and the liberal use to the book. of illustrations and photographic material. Key references providing I would also like to thank Eleanor Lines and Vicki Donald for their more detailed information can be found at the end of each chapter help and patience during the preparation of the material. to assist readers who may wish to obtain more detailed information There may be errors or omissions, and I would welcome any com- about particular topics. ments concerning the book. Readers may also have suggestions for The topics covered are similar to previous editions but several the next edition. I would very much like to hear these and can be chapters have been rewritten by new authors. These include my- contacted at [email protected]. Drew Provan ix