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 |