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Haematology E-Book

Haematology E-Book

Martin R. Howard | Peter J Hamilton

(2013)

Additional Information

Book Details

Abstract

This textbook on haematology in the Illustrated Colour Text series is suitable for medical students, junior doctors and others needing a concise and practical introduction to the subject.

  • Concise and manageable coverage of the major blood diseases and their treatment.
  • Does not overload the reader with science, unlike some competing books.
  • Focus is on the haematology patient and on the practical aspects of the subject.
  • Makes use of the attractive features of the ICT series - double page spreads, lots of colour illustrations, summary boxes.
  • Updating to take account of recenty developments in stem cell biology.
  • Increased emphasis on genomic and proteomic techniques in the diagnosis of haematological malignancy.
  • Use of new imaging techniques including MRI and PET.
  • Description of advances in treatment of leukaemia and lymphoma and in diagnosis and management of patients with thrombophilia and venous thrombosis.
  • Development of new antimicrobial agents in management of immunosuppressed patients with infection.
  • New double page spread on palliative care of patients with blood disorders.
  • New illustrations.

Table of Contents

Section Title Page Action Price
Front cover cover
Haematology i
Copyright page iv
Preface to the fourth edition v
Preface to the first edition v
Acknowledgements v
Table of Contents vi
Anatomy and physiology 2
1 The bone marrow 2
The structure of the bone marrow 2
Haematopoiesis: the stem cell hierarchy 2
Regulators of haematopoiesis 2
2 Red cells 4
Erythropoietin 4
Structure 4
Metabolism 5
Haemoglobin and oxygen transport 5
Ageing and death 5
3 Neutrophils, eosinophils, basophils and monocytes 6
Neutrophils 6
Eosinophils 7
Basophils 7
Monocytes 7
4 Lymphocytes 8
T-lymphocytes 8
B-lymphocytes 8
Natural killer (NK) cells 9
Changes in disease 9
5 The spleen 10
Structure 10
Function 10
Abnormal splenic states 10
Asplenism and hyposplenism 10
Hypersplenism 11
6 Haemostasis 12
The role of platelets 12
Coagulation 12
Regulation of coagulation 13
Fibrinolysis 13
The haematology patient 14
7 History taking 14
History of the presenting complaint 14
Symptoms attributable to anaemia (low haemoglobin concentration) 14
Symptoms attributable to a low white cell count (leucopenia) 14
Symptoms attributable to a low platelet count (thrombocytopenia) 14
Symptoms attributable to abnormal coagulation 14
Symptoms attributable to infiltration by malignancy 14
Systemic enquiry 15
Past medical history 15
Drug history 15
Family history 15
Social history 15
Miscellaneous 15
8 Examining the patient 16
Look at the patient! 16
General examination 16
Examination of the lymph nodes 16
Examination of the spleen 17
9 Laboratory haematology I – Blood and bone marrow 18
The blood count 18
Automated haematology counters 18
The blood film 18
Bone marrow examination 19
Aspirate 19
Trephine biopsy 19
10 Laboratory haematology II – Coagulation and the acute phase response 20
Simple tests of blood coagulation 20
The prothrombin time (PT) 20
Activated partial thromboplastin time (APTT) 20
Quantitation of plasma fibrinogen 20
Measurement of the acute phase response 20
ESR 20
Plasma viscosity 21
C-reactive protein (CRP) 21
Electrophoresis 21
Flow cytometry 21
Anaemia 22
11 Introduction and classification 22
Definition 22
Prevalence 22
General features 22
Classification 22
Morphological classification 22
Aetiological classification 22
Management 23
12 Iron deficiency anaemia 24
Iron 24
Iron deficiency 24
Causes 24
Clinical features 24
Diagnosis 25
The blood count 25
Confirmatory tests 25
Management 25
Investigation of underlying cause 25
Correction of iron deficiency 25
13 Megaloblastic anaemia 26
Why does deficiency of vitamin B12 or folate lead to megaloblastic anaemia? 26
Clinical syndromes 26
Vitamin B12 deficiency 26
Pernicious anaemia 26
Diagnosis 27
Treatment 27
Other causes of vitamin B12 deficiency 27
Folate deficiency 27
14 Haemolytic anaemia I – General features and inherited disorders 28
General features of haemolysis 28
Diagnosis of a haemolytic anaemia 28
Inherited disorders 29
Disorders of the red cell membrane 29
Hereditary spherocytosis 29
Hereditary elliptocytosis 29
Abnormalities of haemoglobin 29
Abnormalities of red cell metabolism 29
Glucose-6-phosphate dehydrogenase (G6PD) deficiency 29
Pyruvate kinase (PK) deficiency 29
15 Haemolytic anaemia II – Acquired disorders 30
Autoimmune haemolytic anaemia 30
Classification 30
Clinical presentation and management 30
Warm autoimmune haemolytic anaemia 30
Cold autoimmune haemolytic anaemia 30
Isoimmune haemolytic anaemia 30
Microangiopathic haemolytic anaemia 31
Haemolytic uraemic syndrome (HUS) 31
Thrombotic thrombocytopenic purpura (TTP) 31
Other acquired haemolytic anaemias 31
16 The thalassaemias 32
Classification 32
Clinical syndromes 32
α-Thalassaemias 32
Hb-Barts hydrops syndrome (– –/– –) 32
HbH disease (–α/– –) 32
α-Thalassaemia traits 32
β-Thalassaemias 32
β-Thalassaemia major 32
Thalassaemia intermedia 33
β-Thalassaemia trait (minor) 33
Prenatal diagnosis 33
17 Sickle cell syndromes 34
Pathophysiology 34
Clinical syndromes 34
Sickle cell anaemia (HbSS) 34
Haemolytic anaemia 34
Vascular-occlusive crises 34
Sequestration crises 34
Other complications 34
Diagnosis 34
Management 35
Prognosis 35
Doubly heterozygous sickling disorders 35
Sickle cell trait (HbAS) 35
Counselling and prenatal diagnosis 35
Screening strategies 35
18 Anaemia of chronic disease 36
Incidence 36
Pathophysiology 36
Diagnosis 36
Management 37
Leukaemia 38
19 Introduction 38
Definition 38
Incidence 38
Aetiology 38
Genetic abnormalities 38
Chromosomal translocations 38
Chromosome deletions and additions 38
Submicroscopic mutations 38
Epigenetic mechanisms 38
Predisposing factors 39
Classification 39
20 Acute myeloid leukaemia 40
Introduction 40
Classification 40
Clinical features 40
Diagnosis 40
Management 41
Supportive care 41
Chemotherapy and stem cell transplantation 41
Prognosis 41
21 Acute lymphoblastic leukaemia 42
Classification 42
Clinical features 42
Diagnosis 42
1. Blood count and film 42
2. Bone marrow aspirate and trephine 42
3. Cytochemistry 42
4. Immunophenotyping 42
5. Cytogenetics 43
6. Molecular techniques 43
Management and outcome 43
General principles 43
ALL in children 43
ALL in adults 43
22 Chronic myeloid leukaemia 44
Pathogenesis 44
Clinical features 44
Diagnosis and monitoring 44
Treatment 45
Chronic phase 45
Advanced disease 45
23 Chronic lymphocytic leukaemia 46
Clinical features 46
Diagnosis 46
Staging 46
Management 46
When to start treatment 46
Choice of treatment 47
24 Other leukaemias 48
Hairy cell leukaemia 48
Clinical features 48
Diagnosis 48
Lymphoma and myeloma 58
29 Hodgkin’s lymphoma 58
Aetiology 58
Classification 58
Clinical presentation 58
Classical Hodgkin’s lymphoma 58
Lymphocyte predominant nodular Hodgkin’s lymphoma 58
Diagnosis and staging 58
Diagnosis 58
Staging 58
Management of classical Hodgkin’s lymphoma 59
Early stage disease 59
Advanced stage disease 59
Prognosis and late effects of treatment 59
30 Non-Hodgkin’s lymphoma 60
Aetiology 60
Classification 60
Clinical presentation 60
Diagnosis and staging 60
Management and prognosis 61
Follicular lymphoma 61
Diffuse large B-cell lymphoma 61
Selected other lymphoma subtypes 61
31 Myeloma 62
Introduction 62
Basic biology 62
Clinical features 62
Diagnosis and staging 62
Management and outcome 63
Drug therapy 63
Management of complications 63
Palliative treatment – a team approach 63
Waldenström’s macroglobulinaemia 63
Myeloproliferative neoplasms 64
32 Polycythaemia 64
Introduction 64
An approach to the patient with polycythaemia 64
Clinical syndromes 64
Polycythaemia vera (PV) 64
Secondary polycythaemia 65
Idiopathic erythrocytosis 65
Apparent polycythaemia 65
33 Essential thrombocythaemia and myelofibrosis 66
Essential thrombocythaemia 66
Clinical features 66
Diagnosis 66
Management 66
Myelofibrosis 66
Clinical features 67
Diagnosis 67
Prognosis and management 67
Haemostasis and thrombosis 68
34 Thrombocytopenia 68
Causes 68
Clinical presentation 68
Clinical syndromes 68
Immune thrombocytopenia (ITP) 68
Acute ITP 68
Chronic ITP 69
Drug-induced thrombocytopenia 69
Post-transfusion purpura 69
35 Disorders of platelet function and vascular purpuras 70
Laboratory testing of platelet function 70
Inherited disorders of platelet function 70
Bernard–Soulier syndrome 70
Glanzmann’s thrombasthenia 70
Other disorders 70
Acquired disorders of platelet function 70
Aspirin 70
Chronic renal failure 71
Cardiopulmonary bypass 71
Haematological diseases 71
Vascular purpuras 71
Inherited disorders 71
Hereditary haemorrhagic telangiectasia (HHT) 71
Inherited diseases of connective tissue 71
Acquired disorders 71
36 Haemophilia 72
Haemophilia A 72
Clinical features 72
Bleeding in haemophilia 72
Complications of treatment 72
Diagnosis 72
Management 72
Treatment of bleeding 72
Treatment of viral infection 73
Gene therapy 73
The carrier state and genetic counselling 73
Haemophilia B 73
37 Von Willebrand disease and other inherited coagulation disorders 74
Von Willebrand disease 74
Classification (Table 37.1 and Fig 37.3) 74
Clinical features 74
Laboratory diagnosis 75
Management 75
Other inherited coagulation disorders 75
Factor deficiencies 75
Factor VIII and factor IX deficiencies 75
Factor XI deficiency 75
Factor VII deficiency 75
Factor V deficiency 75
Factor XIII deficiency 75
Abnormalities of fibrinogen 75
38 Acquired disorders of coagulation 76
Disseminated intravascular coagulation (DIC) 76
Vitamin K deficiency 76
Dietary deficiency 77
Malabsorption 77
In the newborn 77
Liver disease 77
Acquired haemophilia 77
39 Thrombophilia 78
Which patients should be investigated for thrombophilia? 78
Familial thrombophilia 78
Factor V Leiden (FVR506Q) 78
Prothrombin G20210A 78
Protein C and S deficiencies 78
Antithrombin deficiency 79
Other forms of familial thrombophilia 79
Management of familial thrombophilia 79
Acute venous thrombosis 79
Other situations 79
Counselling 79
Acquired forms of thrombophilia 79
Antiphospholipid antibody syndrome 79
Other acquired forms of thrombophilia 79
40 Anticoagulation and thrombolytic therapy 80
Anticoagulation 80
Heparin 80
Unfractionated heparin 80
LMW heparin 80
Warfarin 80
New oral anticoagulant agents 81
Thrombolytic therapy 81
Blood transfusion 82
41 Blood groups and blood testing 82
The blood groups 82
The testing of blood 82
Donor blood 82
Testing before transfusion 82
Blood grouping 82
Antibody screening 83
Crossmatching 83
Practicalities of blood ordering 83
42 Clinical practice 84
Red cell transfusion 84
Practicalities of red cell transfusion 84
Complications of red cell transfusion 84
Immediate 84
Delayed 84
Massive blood transfusion 85
Alternatives to allogeneic blood transfusion 85
Transfusion of platelets and granulocytes 85
Platelet transfusion 85
Granulocyte (neutrophil) transfusion 85
Transfusion of plasma and plasma products 85
Special situations 86
43 The immunosuppressed patient 86
Types of infection 86
Bacteria 86
Fungi 86
Viruses 86
Prevention of infection in the immunosuppressed patient 87
Neutropenia 87
Depressed cell-mediated immunity and hypogammaglobulinaemia 87
Post-splenectomy 87
Treatment of infection 87
The pyrexial neutropenic patient 87
Treatment of specific infections 87
44 Pregnancy 88
Haematological changes 88
Anaemia in pregnancy 88
Thrombocytopenia in pregnancy 88
Incidental (gestational) thrombocytopenia 88
ITP in pregnancy 88
Coagulation abnormalities in pregnancy 89
Thromboembolism and anticoagulant therapy 89
DIC in pregnancy 89
HELLP syndrome 89
45 Paediatric haematology 90
Normal values 90
Neonatal disorders 90
Haemolytic disease of the newborn 90
Diagnosis 90
Management 90
RhD prophylaxis in RhD-negative mothers 90
Anaemia of prematurity 90
Polycythaemia in the neonate 90
Thrombocytopenia in the neonate 91
Iron deficiency in infancy 91
Red cell aplasia in childhood and adolescence 91
Diamond–Blackfan anaemia 91
Transient erythroblastopenia of childhood 91
Congenital dyserythropoietic anaemias (CDAs) 91
46 Haematology in the elderly 92
Haematopoiesis and ageing 92
Anaemia 92
Thrombosis and anticoagulation 92
Haemophilia and other inherited bleeding disorders 92
Haematological malignancy and chemotherapy 93
47 Palliative care in haematological malignancy 94
Palliative chemotherapy and radiotherapy 94
Management of cancer pain 94
Control of non-pain symptoms 94
Psychosocial oncology 95
Complementary therapy 95
48 Systemic disease 96
Renal disease 96
Liver disease and alcohol 96
Malignancy 96
Connective tissue disorders 96
Infections 97
Infectious mononucleosis 97
HIV infection 97
49 The developing world 98
Malaria 98
Pathogenesis 98
Diagnosis 98
Clinical features 98
Non-immune patient 98
Endemic malaria 99
Treatment and prophylaxis 99
Visceral leishmaniasis (kala-azar) 99
Other parasitic diseases detectable in the blood 99
Iron deficiency in hookworm infection 99
Endemic Burkitt’s lymphoma 99
Recent advances in haematology 100
50 Molecular biology 100
Selected techniques used in the analysis of DNA 100
Polymerase chain reaction (PCR) 100
Fluorescence in situ hybridisation (FISH) 100
Comparative genomic hybridisation 100
Microarrays/gene profiling 100
Next-generation sequencing 101
Application of molecular biology in haematology 101
Carrier detection and antenatal detection in genetic disorders 101
Haematological malignancy 101
Diagnosis and classification 101
Minimal residual disease 101
Stem cell transplantation 101
51 Potential advances in treatment 102
Targeting the microenvironment in haematological malignancy 102
New oral anticoagulant drugs 102
Gene therapy 103
Practical procedures 104
52 Venepuncture and venous access 104
Taking a venous blood specimen (venepuncture) 104
Precautions 104
Common problems 104
Children 105
Venous access 105
Peripheral venous cannulation 105
Central venous cannulation 105
53 Bone marrow aspiration and trephine biopsy 106
Bone marrow aspiration 106
Bone marrow trephine biopsy 107
Bone marrow harvesting 107
Appendices 108
Appendix I: Reference ranges in normal adults 108
Blood count 108
Acute phase response 108
Other 108
Appendix II: Selected immunophenotypic (cell surface) markers 109
Appendix III: International prognostic index (IPI) for non-Hodgkin’s lymphoma (NHL) 109
Case histories 110
Case 1 [pp. 24–25] 110
Notes to Case 1 110
Case 2 [pp. 28–29] 110
Notes to Case 2 110
Case 3 [pp. 40–41] 110
Notes to Case 3 111
Case 4 [pp. 46–47] 111
Notes to Case 4 111
Case 5 [pp. 72–73] 111
Notes to Case 5 111
Case 6 [pp. 68–69] 111
Notes to Case 6 111
Case 7 [pp. 86–87] 112
Notes to Case 7 112
Case 8 [pp. 58–59] 112
Notes to Case 8 112
Case 9 [pp. 78–79] 112
Notes to Case 9 112
Case 10 [pp. 52–53] 113
Notes to Case 10 113
Case 11 [pp. 64–65] 113
Notes to Case 11 113
Case 12 [pp. 50–51, 84–85] 113
Notes to Case 12 113
Case 13 [pp. 62–63] 114
Notes to Case 13 114
Case 14 [pp. 34–35] 114
Notes to Case 14 114
Case 15 [pp. 76–77] 114
Notes to Case 15 114
Index 115
A 115
B 116
C 117
D 117
E 118
F 118
G 118
H 119
I 120
J 120
K 120
L 120
M 121
N 122
O 122
P 122
Q 123
R 123
S 123
T 124
U 124
V 124
W 125
Z 125