ABC of Clinical Haematology E-Book
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- Herausgeber: John Wiley & Sons
- Kategorie: Fachliteratur
- Serie: ABC Series
- Sprache: Englisch
ABC of Clinical Haematology An essential guide and introduction to the diagnosis, treatment and management of disorders across the breadth of clinical haematology Extensively revised, this 5th edition of ABC of Clinical Haematology covers all aspects of contemporary haematology, providing the basic science behind the disease and diagnostic aspects, along with up to date management. The text assumes little prior haematology knowledge, enabling clear understanding by non-experts, including medical students and nurses. Full colour illustrations and text boxes highlighting important learning points together enable more efficient reader comprehension. ABC of Clinical Haematology covers: * Iron deficiency anaemia, macrocytic anaemia, hereditary anaemias, polycythaemia vera, essential thrombocythaemia, and myelofibrosis * Chronic myeloid leukaemia, acute leukaemias, lymphoma, stem cell transplantation, cellular therapies, adult myelodysplastic syndrome, and myeloma * Bleeding disorders, thrombosis, anticoagulation, and paediatric haematology, platelet disorders * Amyloidosis, with focus on systemic light chain amyloidosis, and haematological emergencies * New tests, treatments, and management solutions that are now available for certain conditions, especially common blood related disorders With contributions from leading experts in their respective fields, ABC of Clinical Haematology, Fifth Edition provides an ideal reference for primary care practitioners and other healthcare professionals working with patients who have blood related problems. About the ABC series The ABC series has been designed to help you access information quickly and deliver the best patient care, and remains an essential reference tool for GPs, junior doctors, medical students and healthcare professionals. Now offering over 80 titles, this extensive series provides you with a quick and dependable reference on a range of topics in all the major specialties. The ABC series is the essential and dependable source of up-to-date information for all practitioners and students in primary healthcare. To receive automatic updates on books and journals in your specialty, join our email list. Sign up today at www.wiley.com/email
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Veröffentlichungsjahr: 2023
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Table of Contents
Cover
Title Page
Copyright Page
Contributors
Preface to the Fifth Edition
CHAPTER 1: Iron Deficiency Anaemia
Iron metabolism
Clinical features of iron deficiency
Laboratory investigations
Management
Failure to respond to oral iron therapy
Further reading
CHAPTER 2: Macrocytic Anaemia
Vitamin B
12
deficiency: causes, clinical features and consequences
Folate deficiency: causes, clinical features and consequences
Other causes of macrocytosis
Assessment and investigation of macrocytosis
Treatment of vitamin B
12
and folate deficiency
When to refer to haematology
Further reading
CHAPTER 3: The Hereditary Anaemias
The haemoglobinopathies
Further reading
CHAPTER 4: Polycythaemia Vera, Essential Thrombocythaemia and Myelofibrosis
Molecular pathogenesis of MPNs
Erythrocytosis and polycythaemia vera
Thrombocytosis
Primary myelofibrosis
Further reading
CHAPTER 5: Chronic Myeloid Leukaemia
Introduction
Epidemiology
Cytogenetics and molecular biology
Clinical features
Investigations
Management
Further reading
CHAPTER 6: The Acute Leukaemias
Introduction
Subdivision and classification
Epidemiology and aetiology
Clinical features and assessment
Diagnosis and risk stratification
Treatment
Novel strategies
Further reading
CHAPTER 7: Platelet Disorders
Normal haemostasis
Congenital abnormalities
Acquired abnormalities
History and examination of patients
Investigations
Management
Further reading
CHAPTER 8: Adult Myelodysplastic Syndrome
Introduction: what are the myelodysplastic syndromes (MDS)?
Genetic tests in MDS
Deregulation of the immune process
Classification of MDS
How do we predict the outcome of patients with MDS?
Management of MDS
The future of MDS
Further reading
CHAPTER 9: Myeloma and Other Plasma Cell Dyscrasias
Definition and pathogenesis
Multistep pathogenesis of multiple myeloma
Disease complications and their management
Treatment
Other distinct clinical entities of plasma cell dyscrasia
Further reading
CHAPTER 10: Bleeding Disorders, Thrombosis and Anticoagulation
Bleeding disorders
Congenital bleeding disorders
Acquired bleeding disorders
Further reading
CHAPTER 11: Lymphoma
Introduction
Origin
Presentation
Initial investigations
Diagnostic process
Classification of disease
Incidence and survival
Aetiology
Stage and grade
Prognosis
Indicators for treatment
MDT
Primary cutaneous lymphomas
Supportive measures
Response assessment
Relapse
Transformation
Palliative care
Survivorship
Future
Further reading
CHAPTER 12: Stem Cell Transplantation
Introduction
Stem cells
Autologous haematopoietic stem cell transplant
Allogeneic haematopoietic stem cell transplant
Conclusion
Further reading
CHAPTER 13: Cellular Therapies
Introduction
Autologous CAR T cells
T‐cell receptor (TCR) therapy
Tumour‐infiltrating lymphocytes (TILs)
Allogeneic CAR therapy
In vivo CAR T cells
Regulatory T cells
Virus‐specific T cells
Delivery of CAR T‐cell therapy
Future prospects for cellular therapies
Further reading
CHAPTER 14: Paediatric Haematology
Introduction
Investigation of a child with bruising or bleeding
Investigation of a child with anaemia or polycythaemia
Investigation of a child with neutropenia
Childhood congenital bleeding disorders
Childhood leukaemia and lymphoma
Sickle cell disease and thalassaemia
Thalassaemia
Further reading
CHAPTER 15: Amyloidosis with a Focus on Systemic Light Chain Amyloidosis
Introduction
Localised amyloidosis
Systemic amyloidosis
Wild‐type transthyretin amyloidosis and senile systemic amyloidosis
Tissue diagnosis
Cardiac amyloidosis
Treatment for systemic AL amyloidosis
Conclusions
Further reading
CHAPTER 16: Haematological Emergencies
Hyperviscosity syndrome
Tumour lysis syndrome
The vaso‐occlusive pain crisis in sickle cell disease
Spinal cord compression
Bleeding
Thrombotic thrombocytopenic purpura
Infection in patients with impaired immunity
Further reading
Index
End User License Agreement
List of Tables
Chapter 1
Table 1.1 Daily dietary iron requirements.
Table 1.2 Diagnosis of iron deficiency anaemia.
Table 1.3 Characteristics of anaemia associated with other disorders.
Table 1.4 Elemental iron content of various oral iron preparations.
Chapter 2
Table 2.1 Clinical features to assess in suspected vitamin B
12
deficiency. ...
Table 2.2 Laboratory investigations.
Chapter 5
Table 5.1 Definitions of the chronic, accelerated and blastic phases.
Table 5.2 Conventional definitions of cytogenetic responses to treatment fo...
Table 5.3 Milestones for treating chronic myeloid leukaemia expressed as BC...
Table 5.4 Recommended tyrosine kinase inhibitors in case of BCR‐ABL1 resist...
Chapter 6
Table 6.1 Classification of acute leukaemias. Provisional entities are thos...
Table 6.2 Symptoms associated with acute leukaemia according to the bone ma...
Table 6.3 Adverse prognostic factors in ALL.
Table 6.4 European Leukemia Network AML risk factors. Source: Döhner et al....
Chapter 8
Table 8.1 Screening blood tests for patients with potential MDS.
Table 8.2 Top line on molecular genetics in MDS
Table 8.3 Types of MDS: 2016 WHO classification of MDS.
Table 8.4 Revised international Prognostic Scoring System(R‐IPSS) for MDS....
Table 8.5 Risk stratification for R‐IPSS.
Chapter 9
Table 9.1 Diagnostic criteria for monoclonal gammopathy of undetermined sig...
Table 9.2 Smouldering myeloma progression risk.
Table 9.3 Prognostic value of the main chromosomal abnormalities of multipl...
Table 9.4 Laboratory workup for a patient with monoclonal gammopathy.
Table 9.5 Prognostic factors in multiple myeloma.
Chapter 10
Table 10.1 History in a suspected bleeding disorder.
Table 10.2 Investigation in suspected abnormal bleeding.
Table 10.3 Interpretation of abnormal coagulation screen results.
Table 10.4 Prevalence of heritable thrombophilias.
Chapter 11
Table 11.1 Presenting symptoms and signs of lymphomas.
Table 11.2 Approaches to diagnosing lymphoma. This is not an exhaustive lis...
Table 11.3 Features of common and other selected lymphomas.
Table 11.4
(A–E)
Commonly used prognostic scoring systems for patients...
Table 11.5 Summary of indicators for treatment for low‐grade lymphoma and C...
Table 11.6 Summary of treatment modalities used in lymphomas.
Table 11.7 Treatment approaches for common systemic lymphomas.
Table 11.8 Common side effects of treatment.
Table 11.9 Supportive measures.
Chapter 12
Table 12.1 Types of donors for alloHSCT.
Table 12.2 Complications of alloHSCT.
Chapter 13
Table 13.1 FDA/EMA‐approved CAR T‐cell products.
Table 13.2 Complications associated with CAR T cells
Chapter 14
Table 14.1 Disorders identified using neonatal blood‐spot testing.
Table 14.2 Warning signs of primary immunodeficiency (PI) in children.
Table 14.3 Investigations that formulate a specific leukaemia diagnosis.
Table 14.4 Signs suggestive of serious pathology in patient with lymphadeno...
Table 14.5 Thalassaemia complications and their treatment.
Chapter 15
Table 15.1 Common types of systemic amyloidosis.
Table 15.2 Diagnostic and staging investigations for systemic AL amyloidosi...
Table 15.3 Consensus haematological response when assessing patients with s...
Chapter 16
Table 16.1 Symptoms and signs of hyperviscosity.
Table 16.2 Risk stratification for tumour lysis syndrome.
Table 16.3 Sickle cell crises.
Table 16.4 Causes of bleeding according to phase of haemostasis.
Table 16.5 Clinical features of disseminated intravascular coagulation.
Table 16.6 Essential diagnostic investigations for disseminated intravascul...
Table 16.7 Causes of acute disseminated intravascular coagulation.
Table 16.8 Clinical subtypes of thrombotic thrombocytopenic purpura (TTP)....
Table 16.9 Risks of infection in patients with no spleen or hypofunctioning...
List of Illustrations
Chapter 1
Figure 1.1 Diagnosis and investigation of iron‐deficiency anaemia.
Figure 1.2 Blood film showing changes from iron‐deficiency anaemia.
Figure 1.3 Bone marrow aspirate stained with Prussian blue showing adequate ...
Figure 1.4 Intravenous iron infusion. This is usually given on a day ward un...
Chapter 2
Figure 2.1 Bone marrow appearance in megaloblastic anaemia: developing red c...
Figure 2.2 Gastric hydrochloric acid helps release dietary vitamin B
12
, whic...
Figure 2.3 Patient with vitiligo on their neck and back.
Figure 2.4 Diagram demonstrating vitamin B
12
and folate interaction, includi...
Figure 2.5 Blood film in vitamin B
12
showing macrocytic red cells and hypers...
Figure 2.6 Algorithm for treatment for vitamin B
12
deficiency.
Chapter 3
Figure 3.1 Simplified representation of the genetic control of human haemogl...
Figure 3.2 Haemoglobin electrophoresis: (1) normal, (2) newborn, (3) Hb C tr...
Figure 3.3 Peripheral blood film from a patient with sickle cell anaemia sho...
Figure 3.4 Distribution of the thalassaemias (red area).
Figure 3.5 Peripheral blood film in homozygous β thalassaemia showing pronou...
Figure 3.6 Liver biopsy from a patient with β thalassaemia showing pronounce...
Figure 3.7 Inheritance of Hb disease (open boxes represent normal α globin g...
Figure 3.8 Pathophysiology of α thalassaemia.
Chapter 4
Figure 4.1 Raised haematocrit in a patient with polycythaemia vera (left) co...
Figure 4.2 Algorithm for investigation of a patient presenting with a raised...
Figure 4.3 Toe ischaemia in a patient with essential thrombocythaemia.
Figure 4.4 Investigation of thrombocytosis.
Figure 4.5 Bone marrow trephine biopsy from a patient with essential thrombo...
Figure 4.6 Leucoerythroblastic blood film in a patient with primary myelofib...
Figure 4.7 Bone marrow trephine biopsy from a patient with advanced primary ...
Chapter 5
Figure 5.1 The Philadelphia chromosome.
Figure 5.2 Blood film from a patient with chronic myeloid leukaemia.
Chapter 6
Figure 6.1 Symptoms of leukostasis.
Figure 6.2 Classical APML showing characteristic hypergranular promyelocytes...
Figure 6.3 ALL blasts showing typical features of large nucleus, immature ch...
Figure 6.4 Schematic of MRD detection in disease response evaluation.
Chapter 7
Figure 7.1 Normal platelet function.
Figure 7.2 Amegakaryocytic thrombocytopenia with absent radii (TAR syndrome)...
Figure 7.3 Giant granular platelets in peripheral blood film as seen in Bern...
Figure 7.4 Site of abnormality in congenital platelet disorders.
Figure 7.5 Spontaneous skin purpura in severe autoimmune thrombocytopenia.
Figure 7.6 Bone marrow aspirate showing increased megakaryocytes in immune t...
Figure 7.7 Red cell fragmentation in patient who presented with confusion an...
Figure 7.8 Investigation of suspected platelet disorder. PFA, platelet funct...
Chapter 8
Figure 8.1 Algorithm for the diagnosis of adult MDS.
Figure 8.2 Composite images of blood films and bone marrow aspirate from pat...
Figure 8.3 A micromegakaryocyte visualised in the bone marrow aspirate or tr...
Chapter 9
Figure 9.1 Plasma cells adhere to bone marrow stromal cells, resulting in cy...
Figure 9.2 Myeloma‐related complications.
Figure 9.3 Imaging modalities in myeloma: a) plain X‐ray showing pathologica...
Chapter 10
Figure 10.1 A simplified version of the coagulation cascade. TF, tissue fact...
Figure 10.2 Haemophilia arthropathy.
Figure 10.3 Typical diagnostic algorithm for DVT.
Figure 10.4 The natural anticoagulants. APC, activated protein C; AT, antith...
Chapter 11
Figure 11.1 Factors associated with the development of lymphoma. Mos, macrop...
Figure 11.2 Staging of nodal lymphoma using modified Ann Arbor system.
Figure 11.3 Nodal sites used in the FLIPI prognostic scoring system.
Figure 11.4 Mechanisms of treatment for lymphoma.
Chapter 12
Figure 12.1 (a) General timeline of admission for HSCT. (b) The long line us...
Figure 12.2 Trend in (a) numbers of auto/alloHSCT in Europe 1990–2019; b) tr...
Figure 12.3 (a) Indications for total autologous HSCT activity in Europe 201...
Chapter 13
Figure 13.1 Autologous CAR T pathway.
Figure 13.2 Allogeneic CAR products.
Chapter 14
Figure 14.1 Guidance for laboratory investigation of a child with unexplaine...
Figure 14.2 Anaemia differential diagnosis by age.
Figure 14.3 Leukaemia diagnosis emergencies.
Figure 14.4 Suggested management flow diagram for childhood lymphadenopathy....
Figure 14.5 Pathophysiology of sickling.
Figure 14.6 Complications of sickle cell disease.
Chapter 15
Figure 15.1 (a) Macroglossia; (b) nail dystrophy.
Figure 15.2 SAP scintigraphy showing a large amyloid load within the liver, ...
Figure 15.3 Bone marrow trephine with different stains/antibodies/filters (a...
Figure 15.4 Gastrointestinal biopsy with (a) Congo red; (b) apple green bire...
Figure 15.5 (a) ECG showing small QRS complexes; (b) echocardiogram in a pat...
Figure 15.6 Tc‐DPD scintigraphy showing Perugini grade 3 cardiac uptake (Ant...
Chapter 16
Figure 16.1 Blood film in acute myeloid leukaemia. An extremely high number ...
Figure 16.2 (a) Fundal changes in a patient with hyperviscosity (newly diagn...
Figure 16.3 Blood viscosity increases exponentially as the haematocrit rises...
Figure 16.4 Sickled red cells (crescent‐shaped) in homozygous sickle cell di...
Figure 16.5 Sickle cell disease. (a) AP chest radiograph at presentation sho...
Figure 16.6 Magnetic resonance scan of a patient with multiple myeloma showi...
Figure 16.7 Treatment of bleeding in patients receiving anticoagulation.
Figure 16.8 Red cell fragments (irregularly shaped cells amongst the normal ...
Figure 16.9 Temperature chart showing marked swinging pyrexia in a patient w...
Guide
Cover Page
Title Page
Copyright Page
Contributors
Preface to the Fifth Edition
Table of Contents
Begin Reading
Index
WILEY END USER LICENSE AGREEMENT
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ABC of Clinical Haematology
Fifth Edition
EDITED BY
Drew Provan
Emeritus Reader in Autoimmune Haematology, Department of Haematology, Barts and
The London School of Medicine and Dentistry, Queen Mary University of London, London, UK
Claire Harrison
Professor of Myeloproliferative Neoplasms and
Deputy Medical Director, Guy’s and St Thomas’ NHS Foundation Trust UK, London, UK
This fifth edition first published 2023© 2023 John Wiley & Sons Ltd. Published 2023 by John Wiley & Sons Ltd.
Edition HistoryJohn Wiley & Sons Ltd (1e, 1997; 2e, 2003; 3e, 2007; 4e, 2018)
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 law. Advice on how to obtain permission to reuse material from this title is available at http://www.wiley.com/go/permissions.
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Library of Congress Cataloging‐in‐Publication Data applied for
Paperback ISBN: 9781119890744
Cover image: WileyCover design by © rzdeb/Getty Images
Contributors
Michael AustinBarts Health NHS Trust, London, UK; Barts Cancer Institute,Queen Mary University of London, London, UK
Samah BabikerConsultant Haematologists, Guy’s and St Thomas’ NHSFoundation Trust, London, UK
Reuben BenjaminKings College Hospital, London, UK
Catherine BoothBarts Health NHS Trust; NHS Blood and Transplant, London, UK
Michelle Escebedo‐CousinDepartment of Haematology, University College LondonNHS Foundation Trust, London, UK
Anna L. GodfreyDepartment of Haematology and Haemato‐oncology DiagnosticsService, Cambridge University Hospitals NHS Foundation Trust,Cambridge, UK
John D. GraingerRoyal Manchester Children’s Hospital, Manchester, UK
Claire HarrisonProfessor of Myeloproliferative Neoplasms and Deputy MedicalDirector, Guy’s and St Thomas’ NHS Foundation Trust UK,London, UK
Rachel Kesse‐AduClinical Haematology, Guy’s and St Thomas’ NHS FoundationTrust, London, UK
Sally B. KillickUniversity Hospitals Dorset NHS Foundation Trust, RoyalBournemouth Hospital, Bournemouth, UK
Hugues de LavalladeDepartment of Haematological Medicine, Guy’s and St Thomas’NHS Foundation Trust, London, UK
Vickie McDonaldRoyal London Hospital, Barts Health, London, UK
Sally MooreOxford University Hospitals, Oxford, UK; University HospitalsBath, Bath, UK
Igor Novitzky‐BassoHans Messner Allogeneic Transplant Program, Princess MargaretCancer Centre, Toronto, Ontario, Canada
Nita PrasannanConsultant Haematologists, Guy’s and St Thomas’ NHSFoundation Trust, London, UK
Drew ProvanEmeritus Reader in Autoimmune Haematology, Department ofHaematology, Barts and The London School of Medicine andDentistry, Queen Mary University of London, London, UK
Karthik RamasamyOxford University Hospitals, Oxford, UK; Oxford University,Oxford, UK
Lianna ReynoldsRoyal Manchester Children’s Hospital, Manchester, UK
Marie A. ScullyDepartment of Haematology, University College LondonHospitals, London, England; National Institute for HealthResearch Cardiometabolic Programme, University CollegeLondon Hospitals, London, KY
Ayesha Shameem MahmoodNational Amyloidosis Centre, London, UK; Royal Free Hospital,London, UK; UCLH, London, UK
Karen StanleyHaematology Advanced Nurse Practitioner, Clinical Haematology,Guy’s and St Thomas NHS Foundation Trust, London, UK
Vered StaviSoroka Medical Center, Beer Sheva, Israel
Kirsty ThomsonDepartment of Haematology, University College London NHSFoundation Trust, London, UK
John Paul WestwoodDepartment of Haematology, University College LondonHospitals, London, England; Cardiovascular Biomedical ResearchCentre, University College London, London, UK
Bela PatelBarts Health NHS Trust, London, UK; Barts Cancer Institute,Queen Mary University of London, London, UK
David WrenchGuy’s and St Thomas’ NHS Foundation Trust,London, UK
Preface to the Fifth Edition
Haematology has changed hugely in the four years that have elapsed since the fourth edition of the ABC of Clinical Haematology was published. Advances in basic sciences, molecular biology, and therapeutics have led to greater understanding of haematological diseases and provided much‐needed treatments for many malignant and non‐malignant disorders.
As editors, we felt that the ABC of Clinical Haematology required a major overhaul because there have been significant advances in all disease areas, particularly cellular therapies, myeloma, acute leukaemias and non‐malignant disorders of red cells, platelets and bleeding. We have worked with many new authors who have rewritten much of the book, and these revised chapters now reflect modern practice and have brought the book totally up to date. For the first time, we have incorporated a chapter on paediatric haematology, making the book useful to an even wider audience, including medical students, nurses, family doctors and other health professionals involved in the care of adults and children with haematological diseases.
The quality of any book depends on the writing. We are immensely grateful to our colleagues who have contributed valuable chapters despite the pressures of heavy clinical workloads and recent events, which have made things even more stressful.
We are grateful for the project management and support from the team at Wiley, in particular Sophie Bradwell, associate editor; Katherine King, associate managing editor; Tiffany Taylor, copy editor; and many others who have helped us reshape the book and complete the project. We are delighted with the final product, and we sincerely hope readers enjoy the book.
As with all publications, errors can creep in, and we would be happy to hear from readers if any major errors or omissions are found.
