Hoffbrand's Essential Haematology (Essentials)

✍ Scribed by A. Victor Hoffbrand, David P. Steensma

• Publisher: Wiley-Blackwell
• Year: 2019
• Tongue: English
• Leaves: 430
• Edition: 8
• Category: Library

✦ Synopsis

The new and fully updated edition of the definitive haematology textbook for undergraduate and postgraduate students and trainees

Hoffbrand's Essential Haematology is widely regarded as the most authoritative introduction to the subject available, helping medical students and trainee doctors understand the essential principles of modern clinical and laboratory haematology for nearly four decades. Now in its eighth edition, this market-leading textbook introduces the formation and function of blood cells and the diseases that arise from dysfunction and disruption of these processes.

Beautifully presented with over 300 stunning colour illustrations, the new edition has been thoroughly updated to reflect recent advances in knowledge of the pathogenesis of blood diseases and their diagnosis and treatment. This new text:

• Describes disorders and diseases of the blood such as the various anaemias and white cell disorders, leukaemias, lymphomas and myeloma, as well as bleeding and thrombotic disorders
• Incorporates the latest World Health Organization (WHO) classification of haematological neoplastic diseases
• Reviews contemporary application of multiparameter flow cytometry, DNA sequencing and other technologies in evaluating patients with suspected haematological disease
• Discusses the therapeutic use of chimeric antigen T-cells, mono- and bi-specific monoclonal antibodies, inhibitors of intracellular signalling pathways and direct orally acting anticoagulants
• Includes sections on blood transfusion and the haematological aspects of systemic diseases, pregnancy and the neonate

Hoffbrand's Essential Haematology is a vital resource for all students and trainees, and a valuable reference for practicing specialists wishing to update their knowledge.

✦ Table of Contents

Hoffbrand’s Essential Haematology
Contents
Preface to the Eighth Edition
Preface to the First Edition
How to use your textbook
About the companion website
CHAPTER 1 Haemopoiesis
Site of haemopoiesis
Haemopoietic stem and progenitor cells
Bone marrow stroma
The regulation of haemopoiesis
Haemopoietic growth factors
Growth factor receptors and signal transduction
Adhesion molecules
The cell cycle
Transcription factors
Epigenetics
Apoptosis
CHAPTER 2 Erythropoiesis and general aspects of anaemia
Blood cells
Erythropoietin
Indications for erythropoietin therapy
Haemoglobin
Haemoglobin synthesis
Haemoglobin function
Methaemoglobinaemia
The red cell
Red cell metabolism
Red cell membrane
Anaemia
Global incidence
Clinical features of anaemia
Classification and laboratory findings in anaemia
Ineffective erythropoiesis
Assessment of erythropoiesis
CHAPTER 3 Hypochromic anaemias
Nutritional and metabolic aspects of iron
Body iron distribution and transport
Dietary iron
Iron absorption
Iron requirements
Iron deficiency
Clinical features
Causes of iron deficiency
Laboratory findings
Investigation of the cause of iron deficiency (Fig. 3.12)
Treatment
Anaemia of chronic disorders
Iron refractory iron deficiency anaemia (IRIDA)
Sideroblastic anaemia
Lead poisoning
Differential diagnosis of hypochromic anaemia
CHAPTER 4 Iron overload
Assessment of iron status and organ function
Hereditary (genetic, primary) haemochromatosis
African iron overload
Thalassaemia intermedia (non-transfusion-dependent thalassaemia)
Transfusional iron overload
Iron chelation therapy
CHAPTER 5 Megaloblastic anaemias and other macrocytic anaemias
Introduction to macrocytic anaemia
Megaloblastic anaemias
Vitamin B12 (B12, cobalamin)
Absorption
Transport of vitamin B12: the transcobalamins
Biochemical function
Folate
Absorption, transport and function
Biochemical basis for megaloblastic anaemia (Fig. 5.6)
Folate reduction
Causes of severe vitamin B12 deficiency
Causes of mild vitamin B12 deficiency
Pernicious anaemia
Other causes of severe vitamin B12 deficiency
Folate deficiency
Clinical features of megaloblastic anaemia
Vitamin B12 neuropathy (subacute combined degeneration of the cord)
Neural tube defect
Other tissue abnormalities
Laboratory findings
Diagnosis and treatment of vitamin B12 or folate deficiency
Tests for cause of vitamin B12 or folate deficiency
Treatment
Response to therapy
Other megaloblastic anaemias
Abnormalities of vitamin B12 or folate metabolism
Other macrocytic anaemias
Differential diagnosis of macrocytic anaemias
CHAPTER 6 Haemolytic anaemias
Normal red cell destruction
Introduction to haemolytic anaemias
Classification
Clinical features
Laboratory findings
Intravascular and extravascular haemolysis
Hereditary haemolytic anaemias
Membrane defects
Defective red cell metabolism
Acquired haemolytic anaemias
Immune haemolytic anaemias
Red cell fragmentation syndromes
March haemoglobinuria
Infections
Chemical and physical agents
Secondary haemolytic anaemias
CHAPTER 7 Genetic disorders of haemoglobin
Haemoglobin synthesis during human development
Molecular aspects of haemoglobin synthesis
Haemoglobin abnormalities
Thalassaemias
α-Thalassaemia syndromes
β-Thalassaemia syndromes
Non-transfusion-dependent thalassaemia (thalassaemia intermedia): a clinical syndrome
δβ-Thalassaemia
Haemoglobin Lepore
Hereditary persistence of fetal haemoglobin
Association of β-thalassaemia trait with other genetic disorders of haemoglobin
Sickle cell disease
Homozygous disease
Sickle cell trait
Combination of haemoglobin S with other genetic defects of haemoglobin
Prenatal diagnosis of genetic haemoglobin disorders
DNA diagnosis
CHAPTER 8 The white cells, part 1: granulocytes, monocytes and their benign disorders
Granulocytes
Neutrophil (polymorph)
Neutrophil precursors
Monocytes
Eosinophils
Basophils
Granulopoiesis
Control of granulopoiesis: myeloid growth factors
Clinical applications of G-CSF
Monocytes
Disorders of neutrophil and monocyte function
Chemotaxis (cell mobilization and migration)
Phagocytosis
Killing and digestion
Benign disorders
Other rare disorders
Common morphological abnormalities
Causes of neutrophil leucocytosis
The leukaemoid reaction
Leucoerythroblastic reaction
Neutropenia
Benign ethnic neutropenia
Congenital neutropenia
Drug-induced neutropenia
Cyclical neutropenia
Idiopathic and clonal neutropenia of uncertain significance
Autoimmune neutropenia
Diagnosis
Management
Causes of monocytosis, eosinophil and basophil leucocytosis
Monocytosis
Eosinophilic leucocytosis (eosinophilia)
Basophil leucocytosis (basophilia)
Histiocytic and dendritic cell disorders
Dendritic cells
Langerhans’ cell histiocytosis
Haemophagocytic lymphohistiocytosis
Sinus histiocytosis with massive lymphadenopathy
Lysosomal storage diseases
Gaucher disease
Niemann–Pick disease
CHAPTER 9 The white cells, part 2: lymphocytes and their benign disorders
Lymphocytes
B and T lymphocytes
Engineering of T cells, including chimeric antigen receptor T cells
Natural killer cells
Lymphocyte circulation
Immunoglobulins
Antigen–receptor gene rearrangements
Immunoglobulin gene rearrangements
T-cell receptor gene rearrangements
Complement
The immune response
Lymphocytosis
Infectious mononucleosis
Lymphopenia
Immunodeficiency
Differential diagnosis of lymphadenopathy
CHAPTER 10 The spleen
The anatomy and circulation of the spleen
The functions of the spleen
Control of red cell integrity
Immune function
Extramedullary haemopoiesis
Imaging the spleen
Splenomegaly
Tropical splenomegaly syndrome
Hypersplenism
Hyposplenism
Splenectomy
Prevention of infection in hyposplenic patients
CHAPTER 11 The aetiology and genetics of haematological neoplasia
The incidence of haematological neoplasms
The aetiology of haemopoietic neoplasia
Inherited factors
Environmental influences
The genetics of haemopoietic neoplasia
Oncogenes
Tyrosine kinases
Tumour-suppressor genes
Clonal progression
Progression of subclinical clonal haematological mutations to clinical disease
Chromosome nomenclature
Telomeres
Specific examples of genetic abnormalities in haematological neoplasms
Point mutation
Translocations
Deletions
Duplication or amplification
Epigenetic alterations
MicroRNAs
Diagnostic methods used to study neoplastic haemopoietic cells
Karyotype analysis
Fluorescence in situ hybridization analysis
Gene sequencing
DNA microarray platforms
Flow cytometry
Immunohistology (immunohistochemistry)
Circulating neoplastic DNA
Value of genetic markers in management of haematological neoplasia
Initial diagnosis
For establishing a treatment protocol
Monitoring the response to therapy
CHAPTER 12 Management of haematological malignancy
General supportive therapy
Insertion of a central venous catheter
Blood product support (see also Chapter 30)
Haemostasis support
Anti-emetic therapy
Tumour lysis syndrome
Psychological support
Reproductive issues
Nutritional support
Pain
Prophylaxis and treatment of infection
Bacterial infection
Viral infection
Fungal infection
Drugs used in the treatment of haemopoietic malignancies
Cytotoxic drugs (Table 12.2)
Targeted drugs
Other agents
Chimeric antigen receptor (CAR)-T cells
CHAPTER 13 Acute myeloid leukaemia
Classification of leukaemia
Diagnosis of acute leukaemia
Acute myeloid leukaemia (AML)
Pathogenesis
Incidence
Classification
Clinical features
Investigations
Cytogenetics and molecular genetics
Treatment
Outcome
CHAPTER 14 Chronic myeloid leukaemia
Chronic myeloid leukaemia
Clinical features
Laboratory findings
Prognostic scores (stages)
Treatment
CHAPTER 15 Myeloproliferative neoplasms
Genetic drivers of myeloproliferation
Polycythaemia
Classification of polycythaemia
Primary polycythaemia (erythrocytosis): polycythaemia vera (PV)
Clinical features
Laboratory findings
Treatment
Course and prognosis
Congenital causes of primary polycythaemia
Secondary polycythaemia
Apparent polycythaemia
Essential thrombocythaemia
Diagnosis
Clinical and laboratory findings
Prognosis and treatment
Course
Primary myelofibrosis
Clinical features
Laboratory findings
Treatment
Mastocytosis
Chronic neutrophilic leukaemia
Chronic eosinophilic leukaemia
CHAPTER 16 Myelodysplastic syndromes
Myelodysplastic syndromes (MDS)
Pathogenesis
Classification
Clinical features
Laboratory findings
Treatment
Clonal haemopoiesis of indeterminate potential (CHIP)
Myelodysplastic syndromes/myeloproliferative ‘overlap’ neoplasms
Chronic myelomonocytic leukaemia (CMML)
Atypical chronic myeloid leukaemia (aCML)
Juvenile myelomonocytic leukaemia (JMML)
MDS/MPN with ring sideroblasts and thrombocytosis
CHAPTER 17 Acute lymphoblastic leukaemia
Incidence and pathogenesis
Classification
Clinical features
Bone marrow failure
Organ infiltration
Investigations
Cytogenetics and molecular genetics
Treatment
General supportive therapy
Specific therapy of ALL in children
Minimal residual disease
Remission induction
Intensification (consolidation)
Central nervous system-directed therapy
Maintenance
Treatment of BCR-ABL1 positive ALL
Treatment of relapse
Late toxicity
Specific therapy of ALL in adults
Prognosis
CHAPTER 18 The chronic lymphocytic leukaemias
Diagnosis
Monoclonal B-cell lymphocytosis
B-cell diseases
Chronic lymphocytic leukaemia
B-cell prolymphocytic leukaemia
Hairy cell leukaemia
Lymphocytosis in non-Hodgkin lymphomas
T-cell diseases
T-cell prolymphocytic leukaemia
Large granular lymphocytic leukaemia
Adult T-cell leukaemia/lymphoma
CHAPTER 19 Hodgkin lymphoma
History and pathogenesis
Clinical features
Haematological and biochemical findings
Diagnosis and histological classification
Clinical staging
Positron emission tomography
Deauville score
Treatment
Early-stage disease
Advanced-stage disease
Assessment of response to treatment
Relapsed cases
Prognosis
The late effects of Hodgkin lymphoma and its treatment
CHAPTER 20 Non-Hodgkin lymphomas
Introduction to non-Hodgkin lymphomas
Classification
Low- versus high-grade NHL
Pathogenesis
Clinical features of NHL
Investigations
Staging
General principles of treatment of NHL
Specific subtypes of non-Hodgkin lymphoma
Low-grade non-Hodgkin lymphoma
High-grade NHL
T-cell lymphomas
Peripheral T-cell non-Hodgkin lymphoma, unspecified
Angioimmunoblastic lymphadenopathy
Mycosis fungoides
Sézary syndrome
Adult T-cell leukaemia/lymphoma
Enteropathy-associated T-cell lymphomas
Anaplastic large cell lymphoma
Extranodal NK/T cell lymphoma
Histiocytic and dendritic cell neoplasms
Castleman disease
CHAPTER 21 Multiple myeloma and related plasma cell neoplasms
Paraproteinaemia
Multiple myeloma
Pathogenesis
Smouldering myeloma
Diagnosis
Clinical features
Laboratory diagnosis
Treatment
Prognosis
Other plasma cell neoplasms
Solitary plasmacytoma
Plasma cell leukaemia
Osteosclerotic myeloma (POEMS syndrome)
Monoclonal gammopathy of undetermined significance
Amyloidosis
Systemic amyloid light chain amyloidosis
Hyperviscosity syndrome
CHAPTER 22 Aplastic anaemia and bone marrow failure
Pancytopenia
Aplastic anaemia
Pathogenesis
Congenital marrow failure syndromes: Fanconi anaemia
Dyskeratosis congenita
Shwachman–Diamond syndrome
Other congenital marrow failure syndromes
Idiopathic acquired aplastic anaemia
Paroxysmal nocturnal haemoglobinuria
Red cell aplasia
Chronic forms
Transient form
Congenital dyserythropoietic anaemias
Osteopetrosis
CHAPTER 23 Haemopoietic stem cell transplantation
Principles of stem cell transplantation
Collection of stem cells
Stem cell processing
Conditioning
Prevention of graft-versus-host disease
Post-transplant engraftment and immunity
Autologous stem cell transplantation
The human leucocyte antigen system
Human leucocyte antigen and transplantation
Chimerism analysis
Complications
Graft-versus-leukaemia effect and donor leucocyte infusions
Post-transplant lymphoproliferative disease (PTLD)
CHAPTER 24 Platelets, blood coagulation and haemostasis
Platelets
Platelet production
Platelet antigens
Platelet function
Blood coagulation
The coagulation cascade
Coagulation in vivo
Endothelial cells
Haemostatic response
Vasoconstriction
Platelet reactions and primary haemostatic plug formation
Stabilization of the platelet plug by fibrin
Physiological limitation of blood coagulation
Fibrinolysis
Inactivation of plasmin
Tests of haemostatic function
Blood count and blood film examination
Screening tests of blood coagulation
Specific assays of coagulation factors
Tests of platelet function
Tests of fibrinolysis
CHAPTER 25 Bleeding disorders caused by vascular and platelet abnormalities
Abnormal bleeding
Vascular bleeding disorders
Inherited vascular disorders
Acquired vascular defects
Thrombocytopenia
Failure of platelet production
Increased destruction of platelets
Disorders of platelet function
Hereditary disorders
Acquired disorders
Diagnosis of platelet disorders
Treatment of thrombocytopenia and platelet disorders
Thrombomimetics
Platelet transfusions
CHAPTER 26 Coagulation disorders
Hereditary coagulation disorders
Haemophilia A
Von Willebrand disease
Hereditary deficiency of other coagulation factors
Acquired coagulation disorders
Vitamin K deficiency
Liver disease
Disseminated intravascular coagulation
Blood film examination
Coagulation deficiency caused by antibodies
Massive transfusion syndrome (see also p. 385)
Thromboelastography: near-patient (point-of-care) testing
CHAPTER 27 Thrombosis 1: pathogenesis and diagnosis
Arterial thrombosis
Pathogenesis
Clinical risk factors
Venous thrombosis
Pathogenesis and risk factors
Hereditary disorders of haemostasis
Acquired risk factors
Investigation of thrombophilia
Diagnosis of venous thrombosis
Deep vein thrombosis
Pulmonary embolus
Post-thrombotic syndrome (PTS)
CHAPTER 28 Thrombosis 2: treatment
Treatment overview
Venous thromboembolism
Arterial thrombosis
Parenteral anticoagulants
Heparin
Administration and laboratory control
Direct-acting parenteral anticoagulants
Oral anticoagulants
Vitamin K antagonists (warfarin)
Principles of oral anticoagulation with VKAs
Direct oral anticoagulants
Mechanical methods of prophylaxis of DVT and PE
Graduated compression stockings
Intermittent compression devices
Inferior vena cava (IVC) filter
Fibrinolytic drugs
Antiplatelet drugs
P2Y12 inhibitors
Other platelet or vascular active drugs
CHAPTER 29 Haematological changes in systemic diseases
Anaemia of chronic disorders
Haematological problems in the elderly
Anaemia
Thrombosis
Malignant disease (other than primary bone marrow disease)
Anaemia
Polycythaemia
White cell changes
Platelet and blood coagulation abnormalities
Rheumatoid arthritis (and other connective tissue disorders)
Renal failure
Anaemia
Platelet and coagulation abnormalities
Congestive heart failure
Liver disease
Hypothyroidism
Infections
Bacterial infections
Viral infections
Other infections
Bacterial infections
Viral infections
Other infections
Non-specific monitoring of systemic disease
Erythrocyte sedimentation rate
Plasma viscosity
C-reactive protein
CHAPTER 30 Blood transfusion
Blood donor selection
Red cell antigens and blood group antibodies
Blood group antibodies
ABO system
Rh system
Other blood group systems
Hazards of allogeneic blood transfusion
Infection
Prions
Techniques in blood group serology
Cross-matching and pre-transfusion tests
From the patient
From the donor
The cross-match
Electronic cross-match
Complications of blood transfusion
Haemolytic transfusion reactions
Other transfusion reactions
Reduction of blood product use
Blood components
Leucodepletion
Red cells
Autologous donation and transfusion
Granulocyte concentrates
Platelet concentrates
Preparations from human plasma
Fresh frozen plasma
Human albumin solution (4.5%)
Human albumin solution (20%) (salt-poor albumin)
Cryoprecipitate
Freeze-dried factor VIII concentrates
Freeze-dried factor IX–prothrombin complex concentrates
Immunoglobulin
Specific immunoglobulin
Acute blood loss and massive haemorrhage
CHAPTER 31 Pregnancy and neonatal haematology
Haematology of pregnancy
Definition of anaemia in pregnancy
Iron deficiency anaemia
Folate and vitamin B12 deficiency
Thrombocytopenia
Haemostasis and thrombosis
Neonatal haematology
Anaemia in the neonate
Anaemia of prematurity
Neonatal polycythaemia
Neonatal neutropenia
Fetomaternal alloimmune thrombocytopenia
Coagulation
Haemolytic disease of the newborn
Rh haemolytic disease of the newborn
ABO haemolytic disease of the newborn
APPENDIX: 2016 World Health Organization classification of lymphoid and myeloid neoplasms
Index
EULA