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Hoffbrand's Postgraduate Haematology
| Hoffbrand's Postgraduate Haematology |
| Autore | Mead Adam J |
| Edizione | [8th ed.] |
| Pubbl/distr/stampa | Newark : , : John Wiley & Sons, Incorporated, , 2025 |
| Descrizione fisica | 1 online resource (1107 pages) |
| Disciplina | 616.99/418 |
| Altri autori (Persone) |
LaffanMichael A
CollinsGraham P HayDeborah HoffbrandA. Victor |
| Soggetto topico |
Blood Physiological Phenomena
Hematologic Diseases Leukemia - blood Thrombosis - blood |
| ISBN |
9781119706687
1119706688 9781119706670 111970667X 9781119706663 1119706661 |
| Formato | Materiale a stampa  |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Nota di contenuto |
Cover -- Title Page -- Copyright Page -- Contents -- About the editors -- Contributor's list -- Preface to the eighth edition by Adam J Mead -- Preface to the eighth edition by A Victor Hoffbrand -- Preface to the first edition -- About the companion website -- Part I General principles of haematology -- Chapter 1 Human stem cells and haemopoiesis -- Introduction -- Human haemopoietic stem cells and the haemopoietic differentiation hierarchy -- Human haemopoietic stem cell characteristics -- Assays for human haemopoiesis -- Short-term in vitro assays -- Long-term in vitro assays -- Xenotransplantation assay -- Fate-mapping -- Identification of human haemopoietic stem cells -- Development of haemopoietic stem cells -- Regulation of haemopoietic stem cells -- Fate-mapping human haemopoiesis -- Clinical relevance of haemopoietic stem cells -- Selected bibliography -- Chapter 2 Phagocytes -- Introduction -- The phagocyte lineage at a glance -- Phagocyte identification and quantification -- General phagocyte molecules and functions -- Phagocytosis -- Adhesion and chemotaxis -- Pattern recognition receptors and inflammation -- Killing via lytic enzymes and production of reactive radicals -- Phagocyte Particulars -- Neutrophils -- Eosinophils, basophils and mast cells -- Basophils and mast cells -- MPS-Monocytes, macrophages, DCs and multinucleated cells -- Disorders of the phagocyte system -- Neutrophil congenital disorders (Table 2.9) -- Disorders of monocyte/macrophages: a genetic perspective -- Conclusion -- Acknowledgements -- Selected bibliography -- Chapter 3 Erythropoiesis in the human -- Introduction -- The developmental origins of erythropoiesis -- The cellular and molecular pathways leading to definitive erythropoiesis -- Differentiation and terminal maturation.
Changes in the expression of key erythroid proteins during terminal differentiation -- The erythroid niche -- The regulation of erythropoiesis by cytokines and cell signalling -- The regulation of apoptosis in erythropoiesis -- The coordination of erythropoiesis and iron metabolism -- The control of erythropoiesis via the oxygen-sensing system -- The mature red cell, senescence and clearance -- Assessing erythropoiesis -- Concluding remarks -- Selected bibliography -- Chapter 4 Lymphocyte development and function -- Introduction -- B cells -- Development -- Antibodies -- Antibody function -- Fc-Fc receptor-mediated interactions -- Complement fixation -- Neutralising activity -- The B cell receptor -- T cells -- T-cell development -- Antigen recognition by the TCR -- CD4+ and CD8+ T cells and their functions -- Differentiation of primed T cells into effector cells -- Regulatory CD4+ T cells -- The role of co-stimulatory and inhibitory molecules -- Natural killer cells -- Natural killer T cells -- The immune response in cancer and its therapy -- Interpretation of blood lymphocyte counts -- Selected bibliography -- Chapter 5 The spleen -- Structure and function of the spleen -- Splenic blood flow and the red pulp -- Blood pooling within the spleen -- Role of the spleen in ensuring quality control of red cells -- Immunological function of the spleen -- Splenomegaly and hypersplenism -- Spleen size -- Causes of splenomegaly -- Extramedullary haemopoiesis -- Haemolytic anaemia and immune cytopenias -- Infections -- Infiltration by haematological malignancies -- Non-Hodgkin lymphoma -- Hodgkin lymphoma -- Myeloid stem cell disorders -- Infiltration by non-haematological malignancies and tumour-like lesions -- Vascular congestion -- Diagnostic evaluation of splenomegaly and splenic lesions -- Management of splenomegaly -- Post splenectomy sepsis. Patient education -- Vaccination -- Antibiotics -- Changes in peripheral blood following splenectomy -- Leucocyte changes -- Platelet changes -- Hyposplenism -- Selected bibliography -- Chapter 6 Cancer immunotherapy -- Introduction -- Immune evasion and subversion in cancer -- The immune system and tumour microenvironment -- Types of immunotherapy -- Antibody therapy -- Adoptive T cell therapy -- Cancer vaccines -- Cytokine therapy -- Emerging types of immunotherapy -- Allogeneic cell therapies -- Future of immunotherapy -- Selected bibliography -- Chapter 7 Clinical applications of gene editing and therapy -- Introduction -- General principles of gene therapy -- Gene therapy in haemopoietic stem cells -- Gene therapy for thalassaemia -- Gene therapy for sickle cell disease -- Safety and efficacy concerns -- Gene therapy for haemophilia -- Genome editing -- General principles of genome editing -- Delivery of genome editing technology -- Genome editing for the -thalassaemias and sickle cell disease -- Approaches for treating haemoglobinopathies through elevation of fetal haemoglobin levels -- Approaches for treating haemoglobinopathies through direct correction of the gene -- Safety of genome editing -- Conclusion -- Selected bibliography -- Chapter 8 Application of next-generation sequencing in haematology -- Introduction -- NGS technology -- Choice of input substrate -- Choice of assay breadth and depth -- Amplicon versus hybridization panel -- Library preparation -- Short-read technologies -- Long-read technologies -- Bioinformatic processing and interpretation of data -- Bioinformatics pipeline -- Interpretation of variants -- Utility -- Diagnostic -- Prognostic -- Therapeutic -- Measurable residual disease -- Germline predisposition -- Discovery -- Practical considerations -- Cost -- Turn-around-times -- Interpretation complexities. Ethical considerations -- The future -- Selected bibliography -- Chapter 9 The molecular basis of haematological malignancies -- Introduction -- The cancer genome -- Classes of DNA mutations -- Inherited predisposition to haematological cancers -- Acquired DNA mutations in haematological cancers -- Endogenous processes -- Exogenous processes -- From genotype to phenotype -- NOTCH-signalling and lymphoid malignancies -- Epigenetics and leukaemia -- Tyrosine kinases and myeloproliferation -- GM-CSF and RAS signalling in juvenile myelomonocytic leukaemia -- Other Ras pathway mutations in haematological malignancies -- The P53 network -- Splicing factors -- Multiple myeloma -- Clonal evolution and subclonal architecture of haematological cancers -- Clonal evolution of cancer -- Impact of clonal structure on treatment and relapse -- Selected bibliography -- Chapter 10 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 hybridisation -- Molecular genetic analysis -- Whole-genome scanning -- Microarray analysis of gene expression -- Next-generation sequencing -- Laboratory techniques and the WHO classification of tumours of haemopoietic and lymphoid tissues -- Conclusions -- Selected bibliography -- Part II Red blood cell disorders and blood transfusion -- Chapter 11 Iron metabolism, iron deficiency and disorders of haem synthesis -- Introduction -- Distribution of body iron -- Proteins important in iron metabolism -- Haem proteins and iron-containing enzymes -- Ferritin and haemosiderin -- Divalent metal transporter 1 -- Ferroportin -- Other proteins -- Hepcidin -- Erythroferrone -- Matriptase-2. 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 and iron recycling -- Diagnostic methods for investigating iron metabolism -- Serum ferritin -- Bone marrow aspiration -- Serum iron and iron-binding capacity -- Serum transferrin receptors -- Red cell protoporphyrin -- Percentage of hypochromic red cells -- Reticulocyte haemoglobin content -- Iron deficiency -- Absolute iron deficiency -- Iron deficiency anaemia -- Functional iron deficiency -- Anaemia of inflammation -- Tissue effects of iron deficiency -- Causes of absolute iron deficiency -- Causes of anaemia of inflammation -- Features of the anaemia of inflammation -- Management of iron deficiency -- Oral iron therapy -- Parenteral iron therapy -- Pathological alterations in haem synthesis -- Porphyrias -- Congenital erythropoietic porphyria -- Erythropoietic protoporphyria -- Porphyria cutanea tarda -- Lead poisoning -- Sideroblastic anaemia -- Inherited sideroblastic anaemias -- Defective haem synthesis -- Mutations of proteins of the iron-sulfur cluster biogenesis -- Syndromic forms of sideroblastic anaemia -- Defective mitochondria respiratory chain -- Acquired sideroblastic anaemia -- Selected bibliography -- Chapter 12 Iron overload -- Introduction -- Hereditary haemochromatosis -- HFE haemochromatosis -- Body iron quantitation -- Non-HFE haemochromatosis -- Rare genetic causes of iron overload associated with anaemia -- Atransferrinaemia -- DMT1 deficiency -- Acaeruloplasminaemia -- Other causes of iron overload -- Hereditary hyperferritinaemia-cataract syndrome -- Iron-loading anaemias -- Tests of body iron burden -- Serum ferritin -- Liver iron -- Cardiac iron -- Urine iron excretion. Non-transferrin-bound iron. |
| Record Nr. | UNINA-9911019565303321 |
Mead Adam J
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| Newark : , : John Wiley & Sons, Incorporated, , 2025 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
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Postgraduate haematology / / edited by A. Victor Hoffbrand [and three others]
| Postgraduate haematology / / edited by A. Victor Hoffbrand [and three others] |
| Edizione | [Seventh edition.] |
| Pubbl/distr/stampa | Chichester, England : , : Wiley Blackwell, , 2005 |
| Descrizione fisica | 1 online resource |
| Disciplina | 616.1/5 |
| Soggetto topico | Blood - Diseases |
| ISBN |
1-118-85380-6
1-118-85377-6 1-118-85447-0 |
| Formato | Materiale a stampa |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Nota di contenuto |
Intro -- Postgraduate Haematology -- Contents -- Contributor list -- Preface to the seventh edition -- Preface to the first edition -- 1 Stem cells and haemopoiesis -- Introduction -- Hierarchical organization and lineage relationships in the adult haemopoietic system -- Sites of adult haemopoiesis -- Development of HSCs -- Waves of haemopoietic generation in embryonic development -- Embryonic haemopoietic sites and haemopoietic migration -- HSC quiescence, proliferation and ageing -- Haemopoietic-supportive microenvironments -- Adult bone marrow microenvironment -- Microenvironments important for haemopoietic development in the conceptus -- Haemopoietic regenerative and replacement therapies -- Stem cell transplantation -- Gene therapy and gene editing for haemopoietic disease -- New sources of HSCs for transplantation -- Embryonic stem cells and induced pluripotent stem cells -- Selected bibliography -- 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 -- 3 Iron metabolism, iron deficiency and disorders of haem synthesis -- Introduction -- Distribution of body iron -- Proteins important in iron metabolism -- Haem proteins and iron-containing enzymes -- Ferritin and haemosiderin -- Transferrin and transferrin receptors -- Divalent metal transporter 1 -- Ferroportin -- Other proteins -- Hepcidin -- Matriptase-2.
Intracellular iron homeostasis -- Normal iron balance -- Iron absorption -- Dietary and luminal factors -- Mucosal factors: molecular aspects of iron absorption and its regulation -- 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 -- Storage iron -- Iron supply to the tissues -- Iron deficiency anaemia -- Sequence of events -- Causes of iron deficiency (Table 3.4) -- Management of iron deficiency -- Iron refractory iron deficiency anaemia -- Pathological alterations in haem synthesis -- Porphyrias -- Lead poisoning -- Sideroblastic anaemia -- Selected bibliography -- 4 Iron overload -- Introduction -- Hereditary haemochromatosis -- HFE haemochromatosis -- Non-HFE haemochromatosis -- Other causes of iron overload -- Iron-loading anaemias -- Tests of body iron burden -- Iron chelation therapy -- Non-transfusion-dependent thalassaemia (NTDT) -- Acute iron poisoning -- Selected bibliography -- 5 Megaloblastic anaemia -- Introduction -- Underlying basic science -- Biochemical basis of megaloblastic anaemia -- Cobalamin-folate relationship -- Clinical features -- General tissue effects of cobalamin and folate deficiencies -- Neurological manifestations -- Haematological findings -- Peripheral blood -- Bone marrow -- Chromosomes -- Ineffective haemopoiesis -- Cobalamin -- Dietary sources and requirements -- Absorption -- Enterohepatic circulation -- Transport -- Cobalamin analogues -- Causes of cobalamin deficiency -- Diagnosis of cobalamin deficiency -- Tests for the cause of cobalamin deficiency -- Folate -- Dietary folate -- Body stores and requirements -- Absorption -- Enterohepatic circulation -- Transport -- Biochemical functions. Causes of folate deficiency (Table 5.6) -- Diagnosis of folate deficiency -- General management of megaloblastic anaemia -- Treatment of cobalamin deficiency -- Treatment of folate deficiency -- Folinic acid (5-formyl-THF) -- Prophylactic folic acid -- Pregnancy -- Prematurity -- Megaloblastic anaemia not due to cobalamin or folate deficiency or altered metabolism -- Other nutritional anaemias -- Protein deficiency -- Scurvy -- Other deficiencies -- Selected bibliography -- 6 Haemoglobin and the inherited disorders of globin synthesis -- Introduction -- The structure, genetic control and synthesis of haemoglobin -- Genetic control, regulation and synthesis -- Classification of the disorders of haemoglobin -- The thalassaemias and related disorders -- Definition and classification -- The -thalassaemias -- --Thalassaemia and hereditary persistence of fetal haemoglobin -- -Thalassaemia -- The -Thalassaemias -- Thalassaemia intermedia, non-transfusion-dependent thalassaemia -- Screening for thalassaemias -- Structural haemoglobin variants related to thalassaemia (Table 6.3) -- The unstable haemoglobin disorders -- High-oxygen-affinity haemoglobin variants -- Low-oxygen-affinity haemoglobin variants -- Congenital methaemoglobinaemia due to haemoglobin variants -- Acknowledgement -- Selected bibliography -- 7 Sickle cell disease -- Introduction -- Geographic distribution of sickle mutation -- Pathophysiology -- Molecular basis of sickling -- Effect on erythrocytes -- Vaso-occlusion -- Haemolysis -- Clinical manifestations -- Anaemia -- Acute painful episode -- Growth and development -- Infections -- Neurological complications -- Pulmonary complications -- Hepatobiliary complications -- Pregnancy -- Renal complications -- Priapism -- Ocular complications -- Bone complications -- Leg ulcers -- Variant sickle cell syndromes -- Sickle cell trait. HbSC disease -- Sickle cellb-thalassaemia -- Sickle cell anaemia with coexistent -thalassaemia -- Sickle cell/HPFH -- Other sickling syndromes -- Diagnosis -- Peripheral blood findings -- Other laboratory tests -- Haemoglobin electrophoresis -- Other tests to detect sickle haemoglobin -- Newborn screening -- Prenatal diagnosis -- Therapy -- Routine healthcare -- Infections -- Transfusion therapy -- Pain management -- Hydroxycarbamide -- New therapeutic modalities -- Haemopoietic stem cell transplantation -- Psychosocial issues -- Selected bibliography -- 8 Hereditary disorders of the red cell membrane and disorders of red cell metabolism -- Haemolysis -- Definitions -- General features of haemolysis -- Classification -- Red cell membrane disorders -- The red cell membrane -- The integral proteins and vertical interaction -- The clinical phenotypes of hereditary membrane disorders -- Hereditary spherocytosis -- Hereditary elliptocytosis -- Hereditary stomatocytosis and related disorders -- Southeast Asian ovalocytosis -- Abnormalities of membrane lipids -- Defects of red cell metabolism -- The glycolytic pathway (Embden-Meyerhof pathway) -- The Rapoport-Luebering shunt -- Disorders of the glycolytic pathway -- Other defects of the enzymes of the glycolytic system -- Defence against oxidative stress: the production of reducing power -- Pentose phosphate pathway (hexose monophosphate shunt) -- Glucose-6-phosphate dehydrogenase deficiency -- Glutathione -- Nucleotide metabolism -- Pyrimidine 5'-nucleotidase -- Selected bibliography -- 9 Acquired haemolytic anaemias -- Introduction -- Immune haemolytic anaemias -- Autoimmune haemolytic anaemia -- Antibody characteristics and specificity of red cell autoantibodies -- Mechanisms for immune red cell destruction -- Other factors influencing red cell destruction and production. Warm-type autoimmune haemolytic anaemias -- Cold-type autoimmune haemolytic anaemias -- Alloimmune haemolytic anaemia -- Non-immune acquired haemolytic anaemias -- Infections causing haemolytic anaemia -- Fragmentation haemolysis: mechanical haemolytic anaemias -- Chemical and physical agents -- Acquired disorders of the red cell membrane -- Selected bibliography -- 10 Inherited aplastic anaemia/bone marrow failure syndromes -- Introduction -- Fanconi anaemia -- Clinical features -- Cell and molecular biology -- Treatment -- Dyskeratosis congenita -- Clinical features -- Cell biology and link to other diseases -- Treatment -- Shwachman-Diamond syndrome (SDS) -- Clinical features -- Cell and molecular biology -- Treatment -- Diamond-Blackfan anaemia (DBA) -- Clinical features -- Cell and molecular biology -- Treatment -- Congenital dyserythropoietic anaemia (CDA) -- CDA type I -- CDA type II -- CDA type III -- Treatment -- Congenital and cyclical neutropenias -- Thrombocytopenia with absent radii (TAR) -- Congenital amegakaryocytic thrombocytopenia (CAMT) -- Conclusion -- Acknowledgements -- Selected bibliography -- 11 Acquired aplastic anaemia and paroxysmal nocturnal haemoglobinuria -- Acquired aplastic anaemia -- Characterization and definition -- Epidemiology -- Pathogenesis and its clinical relevance -- Detection of somatic mutations in AA -- Clinical features -- Diagnostic investigations and differential diagnosis (Table 11.1) -- Management -- Haemopoietic stem cell transplantation -- Paroxysmal nocturnal haemoglobinuria -- Introduction -- Pathophysiology -- Epidemiology -- Clinical features -- Investigation -- Treatment -- Pregnancy in PNH -- Prognosis -- Future challenges and developments -- Suggested further reading -- Clinical significance of acquired somatic mutations in AA -- Selected bibliography -- 12 Red cell immunohaematology. Introduction. |
| Record Nr. | UNINA-9910131528203321 |
| Chichester, England : , : Wiley Blackwell, , 2005 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
| ||