ABC of Clinical Haematology (eBook)

Drew Provan (Herausgeber)

eBook Download: PDF

2018 | 4. Auflage
John Wiley & Sons (Verlag)
978-1-118-89247-3 (ISBN)

Infos

Thoroughly revised and updated, ABC of Clinical Haematology is an essential guide and introduction to clinical haematology and to the treatment and management of common blood related disorders.

The fourth edition contains new chapters that reflect the most recent developments whilst other chapters have been extensively revised to include the new tests and treatments that are now available for certain conditions such as chronic leukaemia, multiple myeloma and bleeding disorders.

With contributions from leading experts in their respective fields, this text provides an ideal reference for primary care practitioners and other healthcare professionals working with patients who have blood related problems.

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.

Inhalt

Title Page 3
Copyright Page 6
Contents 7
Contributors 9
Chapter 1 Iron-Deficiency Anaemia 11
Iron metabolism 11
Clinical features of iron deficiency 11
Laboratory investigations 13
Management 14
Failure to respond to oral iron therapy 14
Intravenous iron preparations 14
Alternative treatments 15
Prevention 15
Acknowledgements 15
Further reading 15
Chapter 2 Macrocytic Anaemias 17
Deficiency of vitamin B12 (cobalamin) or folate 17
Vitamin B12 deficiency 17
Folate deficiency 18
Consequences of vitamin B12 or folate deficiencies 18
Other causes of macrocytosis 20
Diagnosis 20
Biochemical assays 20
Specialist investigations 20
Autoantibodies 20
Other investigations 21
Treatment 21
Further reading 22
Chapter 3 The Hereditary Anaemias 23
The sickling disorders 23
Classification and inheritance 23
Pathophysiology 23
Geographical distribution 23
Clinical features 23
Diagnosis 24
Prevention and treatment 25
Sickling variants 25
The thalassaemias 25
Classification 25
Distribution 25
Inheritance 25
The ? thalassaemias 26
The ? thalassaemias 27
Prevention and treatment 28
Red cell enzyme defects 28
Red cell membrane defects 28
Other hereditary anaemias 29
Further reading 29
Chapter 4 Polycythaemia Vera, Essential Thrombocythaemia and Myelofibrosis 31
Molecular pathogenesis of myeloproliferative neoplasms 31
Erythrocytosis and polycythaemia vera 32
Polycythaemia vera 32
Secondary erythrocytosis 33
Apparent erythrocytosis 33
Thrombocytosis 34
Essential thrombocythaemia 34
Presentation 34
Laboratory investigations 34
Treatment and prognosis 35
Primary myelofibrosis 35
Presentation 35
Laboratory investigations 35
Treatment and prognosis 36
Further reading 36
Chapter 5 Chronic Myeloid Leukaemia 37
Clinical features 37
Pathophysiology 38
Diagnosis 39
Treatment of chronic-phase disease 39
Imatinib mesylate 40
Monitoring of patients on tyrosine kinase inhibitors 40
Second- and third-generation tyrosine kinase inhibitors 41
Resistance to tyrosine kinase inhibitors 41
Treatment duration and treatment-free remission 41
Advanced-phase disease 41
Further reading 42
Chapter 6 The Acute Leukaemias 43
Introduction 43
Classification 44
Epidemiology 44
Clinical features of acute (myeloid and lymphoblastic) leukaemia 44
Anaemia-related symptoms 44
Infection 45
Bleeding 45
Infiltration 45
Leucostasis 45
Diagnosis of acute (myeloid and lymphoblastic) leukaemia 45
Full blood count 45
Coagulation 45
Biochemistry 45
Bone marrow aspirate and trephine biopsy 45
Immunophenotyping 45
Cytogenetics and molecular studies 46
Lumbar puncture 47
Principles of treatment 47
Supportive care 47
General principles of management 47
Specific management of acute myeloid leukaemia 47
Chemotherapy 47
Role of stem cell transplantation 48
Management of relapse 48
Specific management of acute lymphoblastic leukaemia 48
Chemotherapy 48
Role of stem cell transplantation 48
Novel therapies 48
Further reading 49
Chapter 7 Platelet Disorders 51
Normal haemostasis 51
Congenital abnormalities 52
Fanconi anaemia 52
Thrombocytopenia with absent radii 52
Wiskott–Aldrich syndrome 52
MYH9-related thrombocytopenias 53
Disorders of the surface membrane 53
Platelet storage pool diseases 53
Other conditions 53
Acquired abnormalities 53
Decreased production of platelets 53
Increased consumption of platelets 54
Immune thrombocytopenia 54
Post-transfusion purpura 54
Neonatal alloimmune thrombocytopenia 54
Heparin-induced thrombocytopenia 54
Thrombotic thrombocytopenic purpura 55
Microangiopathic thrombocytopenia 55
Disseminated intravascular coagulation 55
Massive blood transfusion 55
Massive splenomegaly 55
Drugs 55
Bleeding in uraemic patients 55
Essential (primary) thrombocytosis and reactive (secondary) thrombocytosis 55
History and examination of patients 55
Investigations 56
Management 56
Congenital disorders 56
Acquired disorders 57
Further reading 57
Chapter 8 Myelodysplastic Syndromes 59
Introduction 59
Pathogenesis 59
Genetic mutations 59
Deregulation of immune processes 59
Abnormal stem cell environment 60
Spliceosome mutations 60
Therapy-related myelodysplastic syndrome 60
Presentation 60
Investigation 60
Classification 60
Myelodysplastic syndrome associated with isolated 5q? 60
Myelodysplastic/myeloproliferative neoplasms 61
Prognosis 61
Management 62
Supportive care 62
Immunomodulatory agents 64
Hypomethylating agents 64
Conventional chemotherapy 64
Allogeneic stem cell transplantation 64
Future of the myelodysplastic syndromes 64
Further reading 64
Chapter 9 Multiple Myeloma 65
Epidemiology 65
Pathogenesis 65
Clinical features 65
Renal failure 67
Diagnosis 67
Investigations and staging 68
Diagnosis 68
End-organ damage (ROTI) / other complications 68
Other investigations 68
Prognosis 68
Management 69
Transplant-eligible patients 69
Transplant-ineligible patients 69
Treatment at progression 69
Future therapies 69
Monoclonal gammopathy of undetermined significance 69
Other plasma cell disorders 69
Further reading 70
Chapter 10 Bleeding Disorders, Thrombosis and Anticoagulation 71
Bleeding disorders 71
History and examination 71
Laboratory investigation 72
Congenital bleeding disorders 72
Acquired bleeding disorders 73
Venous thromboembolism 73
The inherited thrombophilias 74
Treatment of venous thromboembolism 75
Heparin 75
Warfarin 75
Non-vitamin-K antagonist oral anticoagulants 75
Further reading 75
Chapter 11 Lymphoproliferative Disorders 77
Introduction 77
Aetiology 77
Familial predisposition and geographical factors 77
Immunosuppression 77
Viruses 77
Chronic immune stimulation 77
Presenting features 78
Lymphadenopathy 78
Incidental lymphocytosis 78
Autoimmune phenomena 78
Constitutional symptoms 78
Extranodal disease and organomegaly 78
Bone marrow infiltration 78
Hypercalcaemia 78
Hyperviscosity 78
Diagnosis 78
Blood film morphology 78
Flow cytometry 78
Lymph node biopsy 78
Bone marrow trephine 78
Gene expression profiling with microarray technology 79
Cytogenetics and molecular studies 79
Clonality studies 79
Integrated reporting 79
Staging and prognosis 79
Imaging 80
Bone marrow sampling 80
Prognostic scoring systems 80
Other prognostic markers 81
Treatment 81
Conventional chemotherapy 81
Radiotherapy 81
Central nervous system prophylaxis and treatment 81
Monoclonal antibody therapy 81
Immunomodulatory therapy 81
Transplantation 82
Small molecule B-cell signalling inhibitors 82
Supportive and holistic care 82
Lymphoma subtypes 82
Classification 82
Hodgkin’s lymphoma 82
Diffuse large B-cell lymphoma 83
Follicular lymphoma 83
Burkitt’s lymphoma 83
Chronic lymphocytic leukaemia / small lymphocytic lymphoma 83
Mantle cell lymphoma 83
Peripheral T-cell lymphomas 83
Chapter 12 Stem Cell Transplantation 85
Introduction 85
Stem cells 85
Stem cell collection 85
Autologous stem cell transplantation 86
Allogeneic stem cell transplantation 86
Donor selection 86
Complications of allogeneic stem cell transplantation 87
Infection 87
Graft-versus-host disease 87
Conclusions 89
Further reading 89
Chapter 13 Haematological Problems in Older Adults 91
Introduction 91
Anaemia 91
Iron deficiency 92
Megaloblastic anaemia 92
Anaemia of chronic disease 92
Haematological malignancies 92
Chronic lymphocytic leukaemia 93
Monoclonal gammopathy of undetermined significance 93
Myelodysplastic syndromes 93
Investigation and treatment of suspected haematological malignancies in older people 93
Further reading 94
Chapter 14 Haematological Emergencies 95
Hyperviscosity syndrome 95
Sickle cell crisis 96
Spinal cord compression 97
Bleeding 98
Anticoagulation 98
Thrombocytopenia 99
Disseminated intravascular coagulation 99
Thrombotic thrombocytopenic purpura 100
Infection in patients with impaired immunity 100
Further reading 101
Chapter 15 The Future of Haematology: The Impact of Molecular Biology and Gene Therapy 103
Introduction 103
Gene therapy 103
Genomics 104
Genome editing 105
Immunological modulation without bone marrow transplant 106
Monoclonal antibodies 106
Chimeric antigen receptor T cells 106
Oncolytic viruses 106
Rational drug design 106
Modulation of epigenetic expression 106
Targeting receptor signalling pathways 106
Conclusion 106
Glossary 106
Further reading 107
Index 109
EULA 114

Produkt-Details

• Erscheint lt. Verlag: 1.2.2018
• Reihe/Serie: ABC Series
• Reihe/Serie: ABC Series
• Reihe/Serie: ABC Series
• Sprache: englisch
• Themenwelt: Medizinische Fachgebiete ► Innere Medizin ► Hämatologie
• Schlagworte: ABC of Clinical Haematology • Allgemeine u. Innere Medizin • Allgemeinpraxis, hausärztliche Praxis • Bleeding Disorders, Thrombosis and Anticoagulation • cela Iron deficiency anaemia • Chronic myeloid leukaemia • Doctor • General & Internal Medicine • General Practice/Family Practice • general practitioner</p> • Haematological Emergencies • Haematological problems in older adults • Hämatologie • Hematology • Junior Doctor • <p>clinical haematology • Lymphoproliferative Disorders • Macrocytic anaemias • Medical Science • medical student • Medizin • multiple myeloma • Myelodysplastic Syndromes • platelet disorders • Polycythaemia vera essential thrombocythaemia and myelofibrosis • stem cell transplantation • The Acute Leukaemias • The future of haematology • The Hereditary Anaemias and haematology • the impact of molecular biology and gene therapy
• ISBN-10: 1-118-89247-X / 111889247X
• ISBN-13: 978-1-118-89247-3 / 9781118892473