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Herausgeber: John Wiley & Sons
Kategorie: Fachliteratur
Sprache: Englisch

Beschreibung

This comprehensive textbook is the key resource for postgraduate trainees or residents in haematology. Now in its seventh edition, the book continues to provide everything the reader needs for examination preparation or clinical practice.

Postgraduate Haematology discusses up-to-date knowledge of the pathogenesis, clinical and laboratory features, management and treatment of a wide range of blood and bone marrow disorders in a concise and user friendly style. It presents essential information for everyday use and teaching, as well more detailed scientific background for more in-depth reading, accompanied by thoughtful referencing.

The clearly illustrated full-colour figures and charts demonstrate key facts, and are supplemented by numerous high quality photomicrographs of blood cells and tissues. Over 51 chapters from international authors, including the WHO Classification of Haematopoietic and Lymphoid Tissues, Postgraduate Haematology provides an expert review of malignant and non-malignant haematology.

New sections reflect advances in the specialty, e.g. knowledge gained from new generation sequencing, latest anticoagulant drugs, diagnostic laboratory tools, and treatment strategies
Superb four-color illustrations and photomicrographs of blood cells and tissues throughout
Includes algorithms to aid with decision-making for treatment
Companion website featuring figures and tables from the book

Arm yourself with the textbook of choice for trainees and practitioners in haematology.

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Companion website

This book has a companion website:

www.wiley.com/go/hoffbrand/ph7

with:

Figures and tables from the book for downloading

Postgraduate Haematology

EDITED BY

A Victor Hoffbrand MA, DM, FRCP, FRCPath, FRCP (Edin), DSc, FMedSci

Emeritus Professor of Haematology, University College London, London, UK

Douglas R Higgs MD, FRCP, FRS

Professor of Molecular Haematology & Director,Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK

David M Keeling BSc, MD, FRCP, FRCPath

Consultant Haematologist, Oxford Haemophilia and Thrombosis Centre, Oxford University Hospitals, Oxford, UK

Atul B Mehta MA, MD, FRCP, FRCPath

Consultant Haematologist, Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital, London, UK

Seventh Edition

First published as Tutorials in Postgraduate Haematology © William Heinemann Ltd 1972 Second edition 1981 published © Butterworth Ltd Third edition 1989 published © Butterworth Ltd Fourth edition 1999 published © Butterworth-Heinmann Ltd

Registered office: John Wiley & Sons, Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK

Editorial offices: 9600 Garsington Road, Oxford, OX4 2DQ, UK The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK 111 River Street, Hoboken, NJ 07030-5774, USA

For details of our global editorial offices, for customer services and for information about how to apply for permission to reuse the copyright material in this book please see our website at www.wiley.com/wiley-blackwell

The right of the author to be identified as the author of this work has been asserted in accordance with the UK 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.

Designations used by companies to distinguish their products are often claimed as trademarks. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The publisher is not associated with any product or vendor mentioned in this book. It is sold on the understanding that the publisher is not engaged in rendering professional services. If professional advice or other expert assistance is required, the services of a competent professional should be sought.

The contents of this work are intended to further general scientific research, understanding, and discussion only and are not intended and should not be relied upon as recommending or promoting a specific method, diagnosis, or treatment by health science practitioners for any particular patient. The publisher and the author make no representations or warranties with respect to the accuracy or completeness of the contents of this work and specifically disclaim all warranties, including without limitation any implied warranties of fitness for a particular purpose. In view of ongoing research, equipment modifications, changes in governmental regulations, and the constant flow of information relating to the use of medicines, equipment, and devices, the reader is urged to review and evaluate the information provided in the package insert or instructions for each medicine, equipment, or device for, among other things, any changes in the instructions or indication of usage and for added warnings and precautions. Readers should consult with a specialist where appropriate. The fact that an organization or Website is referred to in this work as a citation and/or a potential source of further information does not mean that the author or the publisher endorses the information the organization or Website may provide or recommendations it may make. Further, readers should be aware that Internet Websites listed in this work may have changed or disappeared between when this work was written and when it is read. No warranty may be created or extended by any promotional statements for this work. Neither the publisher nor the author shall be liable for any damages arising herefrom.

Library of Congress Cataloging-in-Publication Data

Postgraduate haematology / edited by A. Victor Hoffbrand, Douglas R. Higgs, David M. Keeling, Atul B. Mehta. – Seventh edition. p. ; cm. Includes bibliographical references and index. ISBN 978-1-118-85432-7 (cloth) I. Hoffbrand, A. V., editor. II. Higgs, Douglas R., editor. III. Keeling, David (David Michael), editor. IV. Mehta, Atul B., editor. [DNLM: 1. Blood Physiological Phenomena. 2. Hematologic Diseases. 3. Leukemia–blood. 4. Thrombosis–blood. WH 100] RC633 616.1′5–dc23

2015019099

A catalogue record for this book is available from the British Library.

Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.

Cover image: GettyImages-464401418 by Frentusha

Contributor list

Preface to the seventh edition

Preface to the first edition

Chapter 1 Stem cells and haemopoiesis

Introduction

Hierarchical organization and lineage relationships in the adult haemopoietic system

Sites of adult haemopoiesis

Development of HSCs

Haemopoietic-supportive microenvironments

Haemopoietic regenerative and replacement therapies

Selected bibliography

Chapter 2 Erythropoiesis

Introduction

The origins of erythroid cells during development

Specifying the erythroid lineage

Expression of critical transcription factors specifies the erythroid lineage

Terminal maturation of committed erythroid cells

Changes in the expression of transcription factors during terminal maturation

Changes in the expression of erythroid proteins during terminal maturation

Control of erythropoiesis via cell signalling

The erythroid niche

Red cell senescence and clearance

Assessing erythropoiesis

Conclusions

Selected bibliography

Chapter 3 Iron metabolism, iron deficiency and disorders of haem synthesis

Introduction

Distribution of body iron

Proteins important in iron metabolism

Intracellular iron homeostasis

Normal iron balance

Iron absorption

Iron uptake by erythroid cells

Haem synthesis and mitochondrial iron metabolism

Intracellular transit iron and plasma non-transferrin-bound iron

Breakdown of haemoglobin

Diagnostic methods for investigating iron metabolism

Iron deficiency anaemia

Pathological alterations in haem synthesis

Selected bibliography

Chapter 4 Iron overload

Introduction

Hereditary haemochromatosis

Iron-loading anaemias

Selected bibliography

Chapter 5 Megaloblastic anaemia

Introduction

Underlying basic science

Clinical features

Haematological findings

Cobalamin

Folate

General management of megaloblastic anaemia

Prophylactic folic acid

Megaloblastic anaemia not due to cobalamin or folate deficiency or altered metabolism

Other nutritional anaemias

Selected bibliography

Chapter 6 Haemoglobin and the inherited disorders of globin synthesis

Introduction

The structure, genetic control and synthesis of haemoglobin

Classification of the disorders of haemoglobin

The thalassaemias and related disorders

Structural haemoglobin variants related to thalassaemia (Table 6.3)

Acknowledgement

Selected bibliography

Chapter 7 Sickle cell disease

Introduction

Geographic distribution of sickle mutation

Pathophysiology

Clinical manifestations

Variant sickle cell syndromes

Diagnosis

Therapy

Selected bibliography

Chapter 8 Hereditary disorders of the red cell membrane and disorders of red cell metabolism

Haemolysis

Red cell membrane disorders

The clinical phenotypes of hereditary membrane disorders

Defects of red cell metabolism

The glycolytic pathway (Embden–Meyerhof pathway)

Defence against oxidative stress: the production of reducing power

Pentose phosphate pathway (hexose monophosphate shunt)

Glutathione

Nucleotide metabolism

Selected bibliography

Chapter 9 Acquired haemolytic anaemias

Introduction

Immune haemolytic anaemias

Non-immune acquired haemolytic anaemias

Selected bibliography

Chapter 10 Inherited aplastic anaemia/bone marrow failure syndromes

Introduction

Fanconi anaemia

Dyskeratosis congenita

Shwachman–Diamond syndrome (SDS)

Diamond–Blackfan anaemia (DBA)

Congenital dyserythropoietic anaemia (CDA)

Congenital and cyclical neutropenias

Thrombocytopenia with absent radii (TAR)

Congenital amegakaryocytic thrombocytopenia (CAMT)

Conclusion

Acknowledgements

Selected bibliography

Chapter 11 Acquired aplastic anaemia and paroxysmal nocturnal haemoglobinuria

Acquired aplastic anaemia

Paroxysmal nocturnal haemoglobinuria

List of Tables

Chapter 3

Table 3.1

Table 3.2

Table 3.3

Table 3.4

Table 3.5

Table 3.6

Chapter 4

Table 4.1

Table 4.2

Table 4.3

Table 4.4

Chapter 5

Table 5.1

Table 5.2

Table 5.3

Table 5.4

Table 5.5

Table 5.6

Chapter 6

Table 6.1

Table 6.2

Table 6.3

Chapter 7

Table 7.1

Table 7.2

Table 7.3

Table 7.4

Chapter 8

Table 8.1

Table 8.2

Table 8.3

Table 8.4

Table 8.5

Table 8.6

Table 8.7

Table 8.8

Chapter 9

Table 9.1

Table 9.2

Table 9.3

Table 9.4

Table 9.5

Table 9.6

Table 9.7

Chapter 10

Table 10.1

Table 10.2

Table 10.3

Table 10.4

Table 10.5

Table 10.6

Table 10.7

Table 10.8

Table 10.9

Table 10.10

Table 10.11

Table 10.12

Chapter 11

Table 11.1

Table 11.2

Table 11.3

Table 11.4

Chapter 12

Table 12.1

Table 12.2

Table 12.3

Table 12.4

Table 12.5

Chapter 13

Table 13.1

Table 13.2

Table 13.3

Table 13.4

Table 13.5

Table 13.6

Table 13.7

Table 13.8

Table 13.9

Table 13.10

Table 13.11

Table 13.12

Table 13.13

Table 13.14

Table 13.15

Chapter 14

Table 14.1

Table 14.2

Table 14.3

Table 14.4

Table 14.5

Table 14.6

Table 14.7

Table 14.8

Table 14.9

Table 14.10

Table 14.11

Table 14.12

Table 14.13

Chapter 15

Table 15.1

Table 15.2

Chapter 16

Table 16.1

Table 16.2

Table 16.3

Table 16.4

Table 16.5

Table 16.6

Table 16.7

Table 16.8

Table 16.9

Chapter 17

Table 17.1

Table 17.2

Table 17.3

Chapter 18

Table 18.1

Table 18.2

Chapter 19

Table 19.1

Table 19.2

Table 19.3

Chapter 20

Table 20.1

Table 20.2

Table 20.3

Table 20.4

Table 20.5

Table 20.6

Chapter 21

Table 21.1

Table 21.2

Table 21.3

Table 21.4

Chapter 22

Table 22.1

Table 22.2

Table 22.3

Table 22.4

Table 22.5

Chapter 23

Table 23.1

Table 23.2

Table 23.3

Table 23.4

Table 23.5

Table 23.6

Chapter 24

Table 24.1

Table 24.2

Chapter 25

Table 25.1

Table 25.2

Table 25.3

Table 25.4

Table 25.5

Table 25.6

Table 25.7

Table 25.8

Table 25.9

Table 25.10

Table 25.11

Table 25.12

Table 25.13

Table 25.14

Table 25.15

Table 25.16

Table 25.17

Chapter 26

Table 26.1

Table 26.2

Table 26.3

Table 26.4

Table 26.5

Table 26.6

Table 26.7

Table 26.8

Table 26.9

Table 26.10

Table 26.11

Table 26.12

Chapter 27

Table 27.1

Table 27.2

Table 27.3

Table 27.4

Table 27.5

Table 27.6

Table 27.8

Table 27.9

Table 27.10

Table 27.11

Table 27.12

Table 27.13

Chapter 28

Table 28.1

Table 28.2

Table 28.3

Chapter 29

Table 29.1

Table 29.2

Table 29.3

Table 29.4

Table 29.5

Table 29.6

Table 29.7

Table 29.8

Table 29.9

Table 29.10

Chapter 30

Table 30.1

Table 30.2

Table 30.3

Chapter 31

Table 31.1

Table 31.2

Table 31.3

Table 31.4

Table 31.5

Chapter 32

Table 32.1

Table 32.2

Table 32.3

Table 32.4

Table 32.5

Chapter 33

Table 33.1

Table 33.2

Table 33.3

Table 33.4

Table 33.5

Chapter 34

Table 34.1

Table 34.2

Table 34.3

Table 34.4

Table 34.5

Table 34.6

Table 34.7

Table 34.8

Chapter 35

Table 35.1

Table 35.2

Table 35.3

Table 35.4

Table 35.5

Table 35.6

Chapter 36

Table 36.1

Chapter 37

Table 37.1

Chapter 38

Table 38.1

Table 38.2

Table 38.3

Table 38.4

Table 38.5

Chapter 39

Table 39.1

Table 39.2

Chapter 40

Table 40.1

Table 40.2

Table 40.3

Table 40.4

Table 40.5

Table 40.6

Table 40.7

Table 40.8

Table 40.9

Chapter 41

Table 41.1

Table 41.2

Table 41.3

Table 41.4

Table 41.5

Chapter 42

Table 42.1

Table 42.2

Table 42.3

Table 42.4

Table 42.5

Table 42.6

Table 42.7

Table 42.8

Chapter 43

Table 43.1

Table 43.2

Table 43.3

Table 43.4

Table 43.5

Table 43.6

Table 43.7

Chapter 44

Table 44.1

Chapter 45

Table 45.1

Table 45.2

Table 45.3

Table 45.4

Chapter 46

Table 46.1

Table 46.2

Table 46.3

Table 46.4

Table 46.5

Chapter 47

Table 47.1

Table 47.2

Table 47.3

Table 47.4

Chapter 48

Table 48.1

Table 48.2

Table 48.3

Table 48.4

Table 48.5

Table 48.6

Table 48.7

Table 48.8

Table 48.9

Table 48.10

Table 48.11

Table 48.12

Table 48.13

Table 48.14

Table 48.15

Table 48.16

Chapter 49

Table 49.1

Table 49.2

Table 49.3

Chapter 50

Table 50.1

Table 50.2

Table 50.3

Table 50.4

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Contents

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