Oxford Handbook of Clinical Haematology, Second Edition

Oxford Handbook of Clinical Haematology, Second edition Drew Provan, et al. OXFORD UNIVERSITY PRESS 00-Prelims i-viii 10.12.2003 03.51 PM Page i Oxford Handbook of Clinical Haematology 00-Prelims i-viii 10.12.2003 03.51 PM Page ii Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product information and clinical procedures with the most up-to- date published product information and data sheets provided by the manu- facturers and the most recent codes of conduct and safety regulations. The authors 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 work. ᮣ Except where otherwise stated (e.g. Paediatric Haematology), drug doses and recommendations are for the non-pregnant adult who is not breast-feeding. 00-Prelims i-viii 10.12.2003 03.51 PM Page iii Oxford Handbook of Clinical Haematology Second edition Drew Provan Senior Lecturer in Haematology, Barts and The London, Queen Mary’s School of Medicine and Dentistry, University of London Charles R. J. Singer Consultant Haematologist, Royal United Hospital, Bath, UK Trevor Baglin Consultant Haematologist, Addenbrookes NHS Trust, Cambridge, UK John Lilleyman Professor of Paediatric Oncology & Consultant Paediatric Haematologist, Barts and The London, Queen Mary’s School of Medicine and Dentistry, University of London 1 00-Prelims i-viii 10.12.2003 03.51 PM Page iv 1 Great Clarendon Street, Oxford OX2 6DP Oxford University Press is a department of the University of Oxford. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide in Oxford New York Auckland Bangkok Buenos Aires Cape Town Chennai Dar es Salaam Delhi Hong Kong Istanbul Karachi Kolkata Kuala Lumpur Madrid Melbourne Mexico City Mumbai Nairobi São Paulo Shanghai Taipei Tokyo Toronto Oxford is a trade mark of Oxford University Press Published in the United States by Oxford University Press, Inc., New York © Oxford University Press 2004 The moral rights of the author have been asserted Database right Oxford University Press (maker) First edition published 1998 Reprinted 1999, 2000, 2003 Second edition published 2004 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, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, or under terms agreed with the appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, at the address above. This book is sold subject to the condition that it shall not, by way of trade or otherwise, be lent, re-sold, hired out, or otherwise circulated without the publisher’s prior consent in any form of binding or cover other than that in which it is published and without a similar condition including this condition being imposed on the subsequent purchaser. British Library Cataloguing in Publication Data Data available 1 3 5 7 9 10 8 6 4 2 ISBN 0 19 852652 0 Typeset by Drew Provan and EXPO, Malaysia Printed by 00-Prelims i-viii 10.12.2003 03.51 PM Page v Foreword to the first edition The Concise Oxford Dictionary defines a handbook as ‘a short manual or guide’. Modern haematology is a vast field which involves almost every other medical speciality and which, more than most, straddles the worlds of the basic biomedical sciences and clinical practice. Since the rapidly pro- liferating numbers of textbooks on this topic are becoming denser and heavier with each new edition, the medical student and young doctor in training are presented with a daunting problem, particularly as they try to put these fields into perspective. And those who try to teach them are not much better placed; on the one hand they are being told to decongest the curriculum, while on the other they are expected to introduce large slices of molecular biology, social science, ethics and communication skills, not to mention a liberal sprinkling of poetry, music and art. In this over-heated educational scene the much maligned ‘handbook’ could well stage a come-back and gain new respectability, particularly in the role of a friendly guide. In the past this genre has often been viewed as having little intellectual standing, of no use to anybody except the panic- stricken student who wishes to try to make up for months of mis-spent time in a vain, one-night sitting before their final examination. But given the plethora of rapidly changing information that has to be assimilated, the carefully prepared précis is likely to play an increasingly important role in medical education. Perhaps even that ruination of the decent paragraph and linchpin of the pronouncements of medical bureaucrats, the ‘bullet- point’, may become acceptable, albeit in small doses, as attempts are made to highlight what is really important in a scientific or clinical field of enormous complexity and not a little uncertainty. In this short account of blood diseases the editors have done an excel- lent service to medical students, as well as doctors who are not specialists in blood diseases, by summarising in simple terms the major features and approaches to diagnosis and management of most of the blood diseases that they will meet in routine clinical practice or in the tedious examina- tions that face them. And in condensing this rapidly expanding field they have, remarkably, managed to avoid one of the great difficulties and pitfalls of this type of teaching; in trying to reduce complex issues down to their bare bones, it is all too easy to introduce inaccuracies. One word of warning from a battle-scarred clinician however. A précis of this type suffers from the same problem as a set of multiple-choice questions. Human beings are enormously complex organisms, and sick ones are even more complicated; during a clinical lifetime the self-critical doctor will probably never encounter a ‘typical case’ of anything. Thus the outlines of the diseases that are presented in this book must be used as approximate guides, and no more. But provided they bear this in mind, students will find that it is a very valuable summary of modern haematology; the addition of the Internet sources is a genuine and timely bonus. D. J. WEATHERALL April 1998 00-Prelims i-viii 10.12.2003 03.51 PM Page vi Preface to the second edition Haematology has seen many changes since 1998 when the first edition of this small book was written. Most notably, there are major advances in the treatments of malignant blood disorders with the discovery of tyrosine kinase inhibitors which have transformed the outlook for patients with CML, the rediscovery of arsenic for AML and many other new therapies. Progress has been slower in the non-malignant arena since there is still limited evidence on which to base decisions. We have attempted to update each section in the book in order to ensure that it reflects current practice. Although molecular diagnostics have seen huge changes through the Human Genome Project and other methodological developments, we have not included these in great detail here because of lack of space. We have attempted to focus more on clinical aspects of patient care. This edition welcomes two new authors: Professor Sir John Lilleyman, immediate Past-President of the Royal College of Pathologists, is a Paediatric Oncologist at Barts and The London, Queen Mary’s School of Medicine and Dentistry, University of London. John is a leading figure in the world of paediatric haematology with an interest in both malignant and non-malignant disease affecting children. He has extensively revised the Paediatric section of the book, in addition to Immunodeficiency. Dr Trevor Baglin, Consultant Haematologist at Addenbrookes Hospital, Cambridge is Secretary of the British Committee for Standards in Haematology Haemostasis and Thrombosis Task Force. Trevor is the author of many evidence-based guidelines and peer-reviewed scientific papers. He has rewritten the Haemostasis section of the book and brought this in line with modern management. Other features of this edition include the greater use of illustrations such as blood films, marrows and radiological images which we hope will enhance the text and improve readers’ understanding of the subject. We have increased the number of references and provided URLs for key web- sites providing easy access to organisations and publications. There will doubtless be omissions and errors and we take full responsibility for these. We are very keen to receive feedback (good or otherwise!) since this helps shape future editions. If there is something you feel we have left out please complete the Readers comment card. DP CRJS TB JL January 2004 00-Prelims i-viii 10.12.2003 03.51 PM Page vii Preface to the first edition This small volume is intended to provide the essential core knowledge required to assess patients with possible disorders of the blood, organise relevant investigations and initiate therapy where necessary. By reducing extraneous information as much as possible, and presenting key information for each topic, a basic understanding of the pathophysiology is provided and this, we hope, will stimulate readers to follow this up by consulting the larger haematology textbooks. We have provided both a patient-centred and disease-centred approach to haematological disease, in an attempt to provide a form of ‘surgical sieve’, hopefully enabling doctors in training to formulate a differ- ential diagnosis before consulting the relevant disease-orientated section. We have provided a full review of haematological investigations and their interpretation, handling emergency situations, and included the com- monly used protocols in current use on Haematology Units, hopefully providing a unified approach to patient management.

Load more