Oxford handbook of clinical haematology

880 Pages·2009·4.65 MB·English

by  Drew Provan, et al

Haematological emergencies
Septic shock/neutropenic fever b p632
Transfusion reactions b p634
Immediate-type hypersensitivity reactions b p636
Febrile transfusion reactions b p636
Delayed transfusion reaction b p636
Bacterial contamination of blood products b p637
Post-transfusion purpura b p637
Hypercalcaemia b p638
Hyperviscosity b p640
Disseminated intravascular coagulation b p642
Overdosage of thrombolytic therapy b p645
Heparin overdosage b p646
Heparin-induced thrombocytopenia (HIT) b p648
Warfarin overdosage b p650
Massive blood transfusion b p652
Paraparesis/spinal collapse b p654
Leucostasis b p655
Thrombotic thrombocytopenic purpura b p656
Sickle crisis b p658
Tumour lysis syndrome (TLS) b p684
OXFORD MEDICAL PUBLICATIONS
Oxford Handbook of
Clinical
Haematology
Third edition
Published and forthcoming Oxford Handbooks
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Oxford Handbook of Sport and Exercise Medicine
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Oxford Handbook of Urology 2/e
Oxford Handbook of
Clinical
Haematology
THIRD EDITION
Drew Provan
Senior Lecturer in Haematology
Barts and The London School of Medicine and Dentistry
University of London, UK
Charles R.J. Singer
Consultant Haematologist
Royal United Hospital
Bath, UK
Trevor Baglin
Consultant Haematologist
Addenbrookes NHS Trust
Cambridge, UK
Inderjeet Dokal
Professor of Paediatrics
Barts and The London School of Medicine and Dentistry
University of London, UK
1
1
Great Clarendon Street, Oxford OX2 6DP
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Published in the United States
by Oxford University Press Inc., New York
© Oxford University Press, 2009
The moral rights of the author have been asserted
Database right Oxford University Press (maker)
First edition published 1998
Second edition published 2004
Third edition published 2009
All rights reserved. No part of this publication may be reproduced,
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product information and clinical procedures with the most up-to-date published
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and recommendations are for the non-pregnant adult who is not breast-feeding.
v
Preface to the third
edition
It is hard to believe that at least three years have passed since the second
edition of the handbook. As with all medical specialties, Haematology has
seen major inroads with new diagnostic tests, treatments and a plethora
of guidelines. In fact, Haematology has the largest collection of guidelines
covering all aspects of haematology care ((cid:27) http://www.bcshguidelines.
com) and was the fi rst specialty to design guidelines in the 1980s.
The book underwent a major revision with the second edition, most
notably the sections dealing with malignant disease. For the new edition
these have been brought right up to date by Charles Singer. Coagulation
has been entirely rewritten by Trevor Baglin and now truly refl ects the
current investigation and management of coagulation disorders. Following
the retirement of Professor Sir John Lilleyman we needed to fi nd a new
author for the Paediatric Haematology component of the book. Thankfully,
we were able to persuade Professor Inderjeet Dokal to take on this mantle
and he has revised this section thoroughly.
In addition to these signifi cant changes, we have gone through the entire
book and attempted to ensure that obsolete tests have been removed
and that the Handbook, in its entirety, refl ects contemporary haematology
practice.
As ever, we are very keen to hear about errors or omissions, for which
we are entirely responsible! We would also very much like readers to
contact us if there are topics or subject areas which they would like to
see included in the fourth edition (email [email protected]). We also
need more trainee input so if there are any volunteer proof-readers or
accuracy checkers among the haematology trainee community we would
very much like to hear from you.
DP
CRJS
TB
ISD
2008
vi
Preface to the second
edition
Haematology has seen many changes since 1998 when the fi rst 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 thera-
pies. 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 refl ects 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 fi gure 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 Immunodefi ciency. 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 scientifi c 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 fi lms, 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 respon-
sibility for these. We are very keen to receive feedback (good or other-
wise!) 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
2004
vii
Preface to the fi rst
edition
This small volume is intended to provide the essential core knowledge
required to assess patients with possible disorders of the blood, organize
relevant investigations and initiate therapy where necessary. By reducing
extraneous information as much as possible, and presenting key infor-
mation 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 differential
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 unifi ed approach to patient management.
There are additional sections relating to patient support organizations
and Internet resources for further exploration by those wishing to delve
deeper into the subject of blood and its diseases.
Obviously with a subject as large as clinical haematology we have been
selective about the information we chose to include in the handbook. We
would be interested to hear of diseases or situations not covered in this
handbook. If there are inaccuracies within the text we accept full responsi-
bility and welcome comments relating to this.
DP
MC
ASD
CRJS
AGS
1998
viii
Foreword to the fi rst
edition
The Concise Oxford Dictionary defi nes a handbook as ‘a short manual or
guide’. Modern haematology is a vast fi eld 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
proliferating 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 fi elds 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 pan-
icstricken student who wishes to try to make up for months of misspent
time in a vain, one-night sitting before their fi nal 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 scientifi c or clinical fi eld of enor-
mous 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 summarizing 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 fi eld they
have, remarkably, managed to avoid one of the great diffi culties 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, stu-
dents will fi nd 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