Postgraduate Haematology 6th Edition by Hoffbrand AV ISBN 1444323164 9781444323160

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ISBN 10: 1444323164 
ISBN 13: 9781444323160
Author: Hoffbrand AV

Postgraduate Haematology 6th Table of contents:

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
Suggested further reading
Selected bibliography
Chapter 12 Red cell immunohaematology
Introduction
Blood group systems
The red cell membrane and chemistry of blood group antigens
Blood group antibodies
Clinical significance of red cell antibodies
Detection of red cell antigen–antibody reactions
Molecular techniques for blood grouping
The ABO system
The Lewis system
P blood groups
I and i antigens and antibodies
The Rh system
The MNS system
The Kell blood group system
Some other blood group systems
Polyagglutinable red cells
The biological significance of blood group antigens
Selected bibliography
Chapter 13 Clinical blood transfusion
Introduction
Blood transfusion and regulatory aspects
The blood donor (Tables 13.1 and 13.2)
Transfusion-transmitted infection (TTI)
Laboratory tests on blood donations
Clinical and laboratory transfusion practice
Laboratory tests in patients
Special requirements for the selection of blood
Safe administration of blood
Complications of blood transfusion
Haemovigilance
Appropriate use of blood and alternatives to allogeneic blood transfusion
Selected bibliography
Chapter 14 Phagocytes
Introduction
Mechanisms of phagocyte function
Production, structure and dysfunction of phagocytes
Selected bibliography
Chapter 15 Lysosomal storage disorders
Lysosomes
Pathophysiology of lysosomal storage disorders
Prevalence
Diagnosis
General aspects of therapy
Prognosis
Clinical manifestations
Selected bibliography
Chapter 16 Normal lymphocytes and non-neoplastic lymphocyte disorders
Introduction
The anatomy of the immune system
Nature of the antigen-specific receptors on T and B cells
Generation of antigen-specific receptors on T and B lymphocytes
B lymphopoiesis
T-cell production and selection in the thymus
The B-cell repertoire
The T-cell repertoire
Natural killer cells
The immune response
Cytokines and their classification
Chemokines and their classification
Interpretation of blood lymphocyte counts
Infectious mononucleosis
Selected bibliography
Chapter 17 The spleen
Evolution of the spleen
Structure and function
Splenomegaly and hypersplenism
Splenectomy
Hyposplenism
Selected bibliography
Chapter 18 The molecular basis of haematological malignancies
Introduction
The cancer genome
Classes of DNA mutations
Inherited predisposition to haematological cancers
From genotype to phenotype
Clonal evolution and subclonal architecture of haematological cancers
Selected bibliography
Chapter 19 Laboratory diagnosis of haematological neoplasms
Introduction
Blood count and blood film
Bone marrow aspirate
Bone marrow trephine biopsy
Cytochemistry
Histology
Flow cytometric immunophenotyping
Immunohistochemistry
Cytogenetic analysis
Fluorescence in situ hybridization
Molecular genetic analysis
Laboratory techniques and the WHO classification of tumours of haemopoietic and lymphoid tissues
Conclusions
Selected bibliography
Chapter 20 Acute myeloid leukaemia
Disease epidemiology
Pathophysiology and clinical features
Disease classification
Cytogenetics and molecular genetics
Treatment
Factors influencing the risk of relapse
Detection of measurable/minimal residual disease (MRD)
Impact of prognostic factors on treatment choice
Acute promyelocytic leukaemia
Treatment in the older patient
Management of relapse
Future developments
Selected bibliography
Chapter 21 Adult acute lymphoblastic leukaemia
Introduction
Diagnosis of adult ALL
Prognostic and predictive factors in ALL
Initial approach to a patient with ALL
Supportive care (see also Chapter 23)
Specific treatment of ALL
Specific approaches to defined clinical populations in the treatment of ALL
Selected bibliography
Chapter 22 Childhood acute lymphoblastic leukaemia
Introduction
Epidemiology
Aetiology
Pathogenesis (Figure 22.1)
Clinical features
Differential diagnosis
Laboratory features
Immunophenotypic classification (see also Chapter 19)
Cytogenetic and molecular classification
Prognostic factors
Treatment
Future strategies and conclusions
Acknowledgements
Selected bibliography
Chapter 23 Supportive care in the management of leukaemia
Introduction
Psychological
Reproductive
Anaemia
Thrombocytopenia
Infections
Chemotherapy-induced nausea and vomiting (CINV)
Nutritional
Metabolic complications
Skin, nail and dental problems
Pain
Palliation
Selected bibliography
Chapter 24 Chronic myeloid leukaemia
Introduction
Epidemiology and aetiology
Clinical features, natural history, laboratory haematology and cytogenetics
Treatment
Atypical CML/chronic neutrophilic leukaemia (see also Chapters 25 and 26)
Prospects
Acknowledgement
Selected bibliography
Chapter 25 The myelodysplastic syndromes
Introduction
History
Incidence
Aetiology
Classification
Pathogenesis
Immunological abnormalities in MDS
Apoptosis in MDS
Cytogenetic abnormalities in MDS
Molecular basis of MDS
Diagnosis
Natural history and prognostic factors
International Prognostic Scoring System
WHO Classification-based Prognostic Scoring System
Revised International Prognostic Scoring System
Molecular mutations and prognostic scoring systems
Management and treatment
Myelodysplastic/myeloproliferative diseases
Future directions
Selected bibliography
Chapter 26 Myeloproliferative neoplasms
Introduction
The polycythaemias
Essential thrombocythaemia
Primary myelofibrosis
Mastocytosis
Clonal hypereosinophilic syndromes
Chronic neutrophilic leukaemia
Neutrophilic chronic myeloid leukaemia
Transient abnormal myelopoiesis of Down syndrome
Selected bibliography
Chapter 27 Chronic lymphocytic leukaemia and other chronic B-cell disorders
Introduction
General aspects of diagnostic methodology (see also Chapter 19)
Chronic lymphocytic leukaemia
Other B-cell chronic disorders
The leukaemic phase of indolent NHL
Acknowledgements
Selected bibliography
Chapter 28 T-cell lymphoproliferative disorders
Introduction
Chronic T-cell leukaemias
Peripheral T-cell non-Hodgkin lymphomas
Cutaneous T-cell non-Hodgkin lymphomas
Conclusions
Acknowledgements
Selected bibliography
Chapter 29 Multiple myeloma
Definition
Epidemiology and aetiology
Pathogenesis
Differential diagnosis
Other special forms of plasma cell dyscrasia
Disease complications and their management
Prognostic factors
Treatment
Acknowledgements
Selected bibliography
Chapter 30 Amyloidosis
Introduction
Pathogenesis of amyloid
Systemic AL amyloidosis
Localized AL amyloidosis
Other forms of systemic amyloidosis
Conclusion and future directions
Selected bibliography
Chapter 31 The classification of lymphomas: updating the WHO classification
Introduction
Mature B-cell neoplasms
Mature NK-cell/T-cell neoplasms (see also Chapter 28)
Hodgkin lymphoma (see also Chapter 32)
Selected bibliography
Chapter 32 Hodgkin lymphoma
Introduction
Pathological features
Clinical features
Staging and risk stratification
Management
Conventional frontline treatment
Risk-adapted frontline treatment
Relapsed/refractory disease
Treatment of older patients
Late effects
Conclusion
Selected bibliography
Chapter 33 Non-Hodgkin lymphoma: low grade
Introduction
Epidemiology
Histology and classification of low-grade NHL
Follicular lymphoma
Marginal-zone lymphomas
Waldenström macroglobulinaemia
Mantle-cell lymphoma
Selected bibliography
Chapter 34 Non-Hodgkin lymphoma: high grade
Introduction
Epidemiology
Classification of high-grade lymphomas
Aetiology
Molecular basis of lymphomas (see also Chapter 18)
Diagnosis
Staging
Treatment
Diffuse large B-cell lymphoma
Primary mediastinal (thymic) large B-cell lymphoma
Intravascular large B-cell lymphoma
Burkitt lymphoma
B cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma
Transformed lymphomas (see also Chapter 33)
Double-hit or triple-hit lymphomas
DLBCL in HIV-positive patients
Primary central nervous system lymphoma
Post-transplant lymphoproliferative disorder
CNS prophylaxis in high-grade non-Hodgkin lymphoma
Suggested algorithm for therapy of aggressive non-Hodgkin lymphoma (summary)
Rare aggressive T-cell and NK-cell lymphomas (see also Chapter 31)
Future directions
Selected bibliography
Chapter 35 Stem cell transplantation
Immunological basis of stem cell transplantation
Stem cell engraftment
Stem cell mobilization
Conditioning regimens: basic principles
Clinical management of patients undergoing stem cell transplantation
Patient factors determining outcome after allogeneic stem cell transplantation
Indications for transplantation
Factors determining the choice of an allogeneic stem cell donor: the donor algorithm
Management of disease relapse
Future developments in stem cell transplantation
Selected bibliography
Chapter 36 Normal haemostasis
Introduction
Overview of haemostasis
Blood vessels
Coagulation factors
Naturally occurring inhibitors of blood coagulation
Fibrinolysis
Selected bibliography
Chapter 37 The vascular function of platelets
Introduction
Platelet structure and organelles
Animal models
Platelet formation
Thrombus formation
Stimulatory receptors and their signalling pathways
Second messenger pathways underlying activation
The molecular basis of platelet activation
Platelet-based bleeding problems
Platelet function testing
Platelets and thrombosis
Genetics of platelet function disorders
Conclusions and future developments
Acknowledgements
Selected bibliography
Chapter 38 Haemophilia and Von Willebrand Disease
Introduction
Haemophilia
Gene therapy for haemophilia
Haemophilia A and B in females
von Willebrand disease
Selected bibliography
Chapter 39 Rare inherited coagulation disorders
Introduction
Clinical symptoms
Classification
Laboratory diagnosis
Molecular diagnosis
Global haemostasis tests
Treatment
Fibrinogen deficiency
Prothrombin deficiency
Factor V deficiency
Combined deficiency of factor V and factor VIII
Factor VII deficiency
Factor X deficiency
Factor XI deficiency
Factor XIII deficiency
Vitamin-K-dependent coagulation factors deficiency
Concluding remarks
Acknowledgements
Selected bibliography
Chapter 40 Acquired coagulation disorders
Introduction
Tests of coagulation and point-of-care testing
Disseminated intravascular coagulation
Haemostatic dysfunction in acute promyelocytic leukaemia
Vitamin K and related disorders
Haemostatic disturbance in liver disease (Figure 40.5)
Haemostatic disturbance in renal disease
Pregnancy-related haemostatic dysfunction
Haemostatic dysfunction associated with cardiopulmonary bypass surgery
Haemostatic dysfunction associated with trauma
Coagulopathy in massive blood loss
Bruising
Haemostatic dysfunction associated with vasculitis
Arteriovenous malformations
Microthromboembolic disease
Haemostatic dysfunction associated with paraproteinaemia and amyloidosis
Acquired inhibitors of coagulation factors
Selected bibliography
Chapter 41 Congenital platelet disorders
Introduction
Thrombocytopenias
Thrombocytopathies
Treatment
Conclusions
Selected bibliography
Chapter 42 Primary immune thrombocytopenia
Introduction
Clinical features
Reaching a consensus on terminology
Pathophysiology
ITP is multifactorial
T cells may also be involved
Natural history of ITP
Diagnosis
Management
Patients with refractory ITP
TPO receptor agonists
ITP in children
ITP in pregnancy (see also Chapter 50)
Selected bibliography
Chapter 43 Thrombotic thrombocytopenic purpura and haemolytic–uraemic syndrome (congenital and acquired)
Historical introduction
Thrombotic thrombocytopenic purpura
Haemolytic uraemic syndrome
Concluding remarks
Selected bibliography
Chapter 44 Heritable thrombophilia
Introduction
Heritable thrombophilias associated with venous thrombosis
Treatment of patients with venous thrombosis and heritable thrombophilias
Case finding
Prevention of thrombosis associated with oestrogen-containing hormone preparations
Prevention of pregnancy-associated venous thrombosis
Purpura fulminans
Vitamin K antagonist-induced skin necrosis
Thrombophilia and arterial thrombosis
Neonatal stroke (see also Chapter 50)
Laboratory methodology and testing strategy
Counselling and genetic testing
Selected bibliography
Chapter 45 Acquired venous thrombosis
Epidemiology of venous thromboembolism (VTE)
Pregnancy and venous thromboembolism
Immobility as a risk factor for venous thromboembolism
Iatrogenic venous thromboembolism
Antiphospholipid syndrome
Venous thromboembolism and cancer
Thrombotic risk in myeloproliferative disease (polycythaemia rubra vera and essential thrombocythaemia) (see also Chapter 26)
Inflammation and thrombosis
Haematological prothrombotic states due to non-malignant diseases of the blood and bone marrow
Selected bibliography
Chapter 46 Antithrombotic agents
Heparins
Direct thrombin inhibitors
Vitamin K antagonists (VKA)
Non-vitamin K antagonist oral anticoagulants (NOACs)
Antiplatelet drugs
Selected bibliography
Chapter 47 Management of venous thromboembolism
Introduction
Diagnosis of deep vein thrombosis
Diagnosis of pulmonary embolism
Treatment of VTE
Long-term complications of VTE
Risk of recurrence and duration of anticoagulant therapy
Other treatments
Venous thrombosis in unusual sites
Selected bibliography
Chapter 48 Haematological aspects of systemic disease
Anaemia of chronic disease
Malignancy
Connective tissue disorders (Table 48.8)
Renal disease (Table 48.9)
Endocrine disease (Table 48.10)
Liver disease (Table 48.11)
Infections (Tables 48.12 and 48.13)
Haematological aspects of pregnancy (Table 48.15)
Anaemia in the elderly
Haematological aspects of HIV infection (Table 48.16)
Selected bibliography
Chapter 49 Haematological aspects of tropical diseases
Introduction
Ethnic variations in reference ranges
Tropical diseases with organisms in peripheral blood or bone marrow
Tropical diseases associated with changes in FBC and / or coagulation
Non-specific haematological abnormalities associated with tropical diseases
Selected bibliography
Chapter 50 Neonatal haematology
Developmental haemopoiesis
Neonatal anaemia
White cell disorders
Haemostasis and thrombosis in the newborn
Selected bibliography
Chapter 51 WHO Classification: Tumours of the Haematopoietic and Lymphoid Tissues (2008)
Myeloproliferative neoplasms
Myelodysplastic/myeloproliferative neoplasms
Myelodysplastic syndromes
Acute myeloid leukaemia
Precursor lymphoid neoplasms
Mature B-cell neoplasms
Mature T-cell and NK-cell neoplasms
Hodgkin lymphoma
Histiocytic and dendritic cell neoplasms
Post-transplant lymphoproliferative disorders

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