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per Medici e Operatori Sanitari
per Medici e Operatori Sanitari
LIBRERIA STUDIUM
Color Atlas of Clinical Hematology: Molecular and Cellular Basis of Disease, 5th Edition
Hoffbrand, Vyas, Campo, Haferlach, Gomez
Editore
Wiley
Anno
2019
Pagine
600
ISBN
9781119057017
192,00 €
DA SCONTARE
Provides coverage of the pathogenesis, clinical, morphologic, molecular and investigational aspects of a full range of blood disorders seen in daily practice
The revised fifth edition of this renowned atlas presents readers with a comprehensive, visual guide to clinical hematology, featuring 2700 full-color photographs and figures depicting the spectrum of hematological diseases. Ranging from photographs of the clinical manifestations and key microscopic findings to diagrams of the molecular aspects of these diseases, the book provides up-to-date information of the blood diseases that clinicians encounter every day.
Color Atlas of Clinical Hematology: Molecular and Cellular Basis of Disease offers the reader an understanding of normal cell machinery, and of the molecular basis for such processes as DNA and cell replication, RNA species, trafficking and splicing, protein synthesis, transcription factors, growth factor signal transduction, epigenetics, cell differentiation, autophagy, and apoptosis. The text goes on to explore how these processes are disturbed in the various diseases of the bone marrow, blood, and lymphoid systems.
- Helps solve difficult diagnostic challenges and covers complex principles using highly illustrative, full-color images
- Explores all aspects of benign and malignant hematology, including blood transfusion and coagulation with extensive coverage of the pathogenesis of common clinical entities
- Provides a quick and easy reference of key diagnostic issues in a comprehensive yet concise format
- Includes and illustrates the WHO Classification of Hematologic Malignancies
- Illustrates the new knowledge of the molecular basis of inherited and acquired blood diseases
Color Atlas of Clinical Hematology: Molecular and Cellular Basis of Disease is the must-have resource for both trainee and practising hematologists, and for every department of hematology.
Preface xi
1 Molecular Biology of the Cell 1
Compartmentalization of the Cell 1
Gene Transcription and Messenger RNA Translation: The Production and Journey of mRNA 3
DNA Mutations can Alter Protein Synthesis by a Number of Mechanisms 6
Transcriptional Control of Gene Expression 6
Transcription Factors, Control of Gene Expression, and Lineage Commitment 14
Micro‐RNAs 16
Regulatory Noncoding RNAs 16
DNA Replication and Telomeres 16
Mutations and How They Result in Disease 17
Cell Cycle 19
Apoptosis 20
Organelles in Cells 21
Link Between Metabolism and Gene Expression 22
Removal Of Circulating and Cellular Debris by Lysosomes 26
Protein Ubiquitination 26
2 Hematopoiesis 27
Sites of Hematopoiesis 27
Road Maps of Hematopoiesis 27
Transcriptional Control of Hematopoiesis 32
The Hematopoietic Niche 33
3 Growth Factor Signaling 37
Signaling at Different Stages of Hematopoiesis 37
Cytokine Receptors 38
Signaling Pathways Downstream of Receptors 39
Mutations in Signaling Components Leading to Clonal Hematologic Disorders 46
4 Erythropoiesis and Examination of the Peripheral Blood and One Marrow 47
Erythropoiesis 47
Examination of Peripheral Blood and the Bone Marrow 49
Erythroid Cells in the Bone Marrow and Peripheral Blood 49
5 Hypochromic Anemias 53
Iron Metabolism 53
Iron Absorption 55
Hepcidin 55
Iron Homeostasis 55
Iron‐Deficiency Anemia 55
Sideroblastic Anemia 62
Alcohol 67
Lead Poisoning 67
Differential Diagnosis of Hypochromic Microcytic Anemias 68
6 The Porphyrias and Iron Overload 69
Congenital Erythropoietic Porphyria 69
Congenital Erythropoietic Protoporphyria 71
Iron Overload 71
7 Megaloblastic Anemias 76
Clinical Features 77
Bone Marrow Appearances 81
Causes of Megaloblastic Anemia 81
8 Hemolytic Anemias 89
Hereditary Hemolytic Anemia 91
Acquired Hemolytic Anemia 99
9 Genetic Disorders of Hemoglobin 106
Thalassemia 106
α‐Thalassemia 118
Structural Hemoglobin Variants 122
10 Benign Disorders of Phagocytes 130
Granulopoiesis and Monocyte Production 130
Neutrophils (Polymorphs) 131
Mononuclear Phagocytic System 133
Reticuloendothelial System 133
Hereditary Variation in White Cell Morphology 137
Disorders of Phagocytic Function 140
Leukocytosis 142
Leukoerythroblastic Reaction 145
Neutropenia 146
Myelokathexis 149
Lysosomal Storage Diseases 150
11 Benign Disorders of Lymphocytes and Plasma Cells 155
T Cells 155
B Cells, 157
Natural Killer Cells 160
Lymphocyte Proliferation and Differentiation 162
Somatic Hypermutation In Normal B Cells 164
Lymphocyte Circulation 164
Complement 165
Lymphocytosis 165
Lymphadenopathy 167
Primary Immunodeficiency Disorders 168
12 Aplastic and Dyserythropoietic Anemias 185
Aplastic Anemia 185
Red Cell Aplasia 193
Congenital Dyserythropoietic Anemias 195
13 The Hematologic Neoplasms:Laboratory Techniques And Acute Myeloid Leukemia 198
Diagnostic Techniques 198
Acute Myeloid Leukemia 208
14 Acute Lymphoblastic Leukemia 241
Classification 241
Clinical Features 242
Microscopic Appearances 244
Immunology 246
Cytogenetics 247
Fluorescence in Situ Hybridization 248
Molecular Findings 248
Minimal Residual Disease 250
15 Myelodysplastic Syndromes 256
Clinical Features 256
Molecular Genetics 263
Mirage Syndrome 268
Clonal Hematopoiesis of Indeterminate Potential 269
16 Myeloproliferative Neoplasms 271
Chronic Myeloid Leukemia, BCR‐ABL1+ 271
Chronic Neutrophilic Leukemia 277
The Nonleukemic Myeloproliferative Diseases 277
Leukemic Transformation of Polycythemia Vera and Myelofibrosis 295
Chronic Eosinophilic Leukemia, Not Otherwise Specified 300
Myeloproliferative Disorder Unclassifiable 300
17 Mastocytosis, Myeloid/Lymphoid Neoplasms With Eosinophilia and Specific Cytogenetic Rearrangements, Myelodysplastic/Myeloproliferative Neoplasms 302
Mastocytosis 302
Paraneoplastic Pemphigu 309
Myeloid/Lymphoid Neoplasms with Eosinophilia and Abnormalities of PDGFRA,PDGFRB or FGFR1, or with PCM1‐JAK2 309
Myelodysplastic/Myeloproliferative Neoplasms With Ring Sideroblasts and Thrombocytosis,312
Juvenile Myelomonocytic Leukemia 314
18 Chronic Lymphocytic Leukemia and Other Mature B‐ and T‐Cell Leukemias 317
Mature B‐Cell Leukemias 317
Mature C-Cell Leukemias 328
19 Small B‐Cell Lymphomas 335
Epidemiology 336
Etiologic Factors 336
Genetic and Molecular Abnormalities 337
Clinical Features and Diagnosis 337
Imaging 341
Diagnosis 342
Lymphoplasmacytic Lymphoma/Waldenstrom Macroglobulinemia 345
Monoclonal Gammopathy of Undetermined Significance IgM+ 346
Heavy Chain Diseases 346
Splenic Marginal Zone Lymphoma 348
Extranodal Marginal Zone Lymphoma of Mucosa‐Associated Lymphoid Tissue (Malt Lymphoma) 349
Nodal Marginal Zone B‐Cell Lymphoma 351
Follicular Lymphoma 352
Mantle Cell Lymphoma 356
20 Aggressive Mature B‐Cell Neoplasms 361
Diffuse Large B‐Cell Lymphoma, Nos 361
T‐ Cell/Histiocytic‐Rich Large B‐Cell Lymphoma 365
Primary Cutaneous Diffuse Large B‐Cell Lymphoma, Leg Type 365
Lymphomatoid Granulomatosis, 365
Primary Mediastinal (Thymic) Large B‐Cell Lymphoma 365
Intravascular Large B‐Cell Lymphoma 366
ALK‐Positive Diffuse Large B‐Cell Lymphoma, 366
Plasmablastic Lymphoma 370
Primary Effusion Lymphoma and Other HHV8‐Related Disorders, 370
Burkitt Lymphoma 371
High Grade B‐Cell Lymphoma 372
21 Myeloma and Related Neoplasms 376
Multiple (Plasma Cell) Myeloma 376
Other Plasma Cell Tumors 383
Hyperviscosity Syndrome 384
Other Causes of Serum M‐Proteins 384
Amyloidosis 389
22 Peripheral T‐ and Nk‐Cell Neoplasms 399
Mature T‐ and NK‐Cell Neoplasm 399
Epstein– Barr Virus Positive T‐Cell Lymphoproliferative Diseases of Childhood 399
Extranodal NK‐/T‐Cell Lymphoma, Nasal Type 400
Enteropathy‐Associated T‐Cell Lymphoma 401
Hepatosplenic T‐Cell Lymphoma 402
Primary Cutaneous T‐Cell Lymphomas 404
Subcutaneous Panniculitis‐Like T‐Cell Lymphoma 404
Mycosis Fungoides 405
Sezary Syndrome 407
Primary Cutaneous CD30+ T‐Cell Lymphoproliferative Disorders 408
Primary Cutaneous γδ T‐Cell Lymphoma 410
Primary Cutaneous Aggressive Epidermotrophic CD8+ T‐Cell Lymphoma (Provisional Category) 410
Primary Cutaneous Small/Medium CD4+T‐Cell Lymphoproliferative Disorder (Provisional Category) 411
Peripheral T‐Cell Lymphoma, Not Otherwise Specified 411
Angioimmunoblastic T‐Cell Lymphoma 412
Anaplastic Large Cell Lymphoma, ALK Positive 413
Anaplastic Large Cell Lymphoma, ALK Negative 414
23 Hodgkin Lymphoma 418
Presentation and Evolution 418
Histology 418
Classification of Hodgkin Lymphoma 421
Staging Techniques 424
Prognostic Factors 432
24 Histiocytic Disorders 434
Hemophagocytic Lymphohistiocytosis (Hemophagocytic Syndrome) 434
Xanthogranuloma 435
Rosai–Dorfman Disease 435
Histiocytic and Dendritic Cell Neoplasms 439
Histiocytic Sarcoma 439
Langerhans Cell Histiocytosis 439
Langerhans Cell Sarcoma 444
Indeterminate Dendritic Cell Tumor 444
Interdigitating Dendritic Cell Sarcoma 444
Follicular Dendritic Cell Sarcoma 446
Fibroblastic Reticular Cell Tumor 447
Disseminated Juvenile Xanthogranuloma 447
Erdheim–Chester Disease 447
Blastic Plasmacytoid Dendritic Cell Neoplasm 448
25 Stem Cell Transplantation 451
Human Leukocyte Antigen System 451
Other Human Leukocyte Antigens 452
Stem Cell Transplantation 452
26 Normal Hemostasis,Platelet Production and Function 468
The Coagulation Cascade 470
Regulation of Coagulation 470
Megakaryocyte and Platelet Production 472
Platelet and Von Willebrand Factor Function 475
27 Vascular and Platelet Bleeding Disorders 479
Vascular Bleeding Disorders 479
Platelet Bleeding Disorders 480
28 Inherited and Acquired Coagulation Disorders 494
Hereditary Coagulation Disorders 494
Acquired Coagulation Disorders 504
29 Thrombosis and Antithrombotic Therapy 510
Atherothrombosis 510
Venous Thrombosis 510
Acquired Risk Factors For Venous Thrombosis 515
Antiphospholipid Syndrome 515
Diagnosis of Venous Thrombosis 515
Diagnosis of Pulmonary Embolus 516
Antiplatelet Drugs 519
Anticoagulant Therapy 520
Fibrinolytic Agents 523
Post‐Thrombotic Syndrome 527
30 Hematologic Aspects of Systemic Diseases 528
Anemia of Chronic Disorders 528
Malignant Diseases (Other than Leukemias,Lymphomas, Histiocytic and Myeloproliferative Disorders) 529
Rheumatoid Arthritis and Other Connective Tissue Diseases 530
Renal Failure 533
Liver Disease 533
Hypothyroidism 538
Infections 539
Granulomatous Inflammation 542
Osteopetrosis (Albers–Schonberg or Marble Bone Disease) 543
Anorexia Nervosa 544
Cystinosis 544
Primary Oxaluria 547
31 Parasitic Disorders 548
Malaria 548
Toxoplasmosis 552
Babesiosis 552
Trypanosomiasis 553
Bancroftian Filariasis 553
Loiasis 554
Bartonellosis 554
Relapsing Fever 555
32 Blood Transfusion 556
Red Cell Antigens 556
Red Cell Antibodies 557
ABO System 557
Rh System 558
Blood Grouping and Cross‐Matching 559
Red Cell Components 559
Clinical Blood Transfusion 560
Complications of Blood Transfusion 560
Other Blood Components 564
Appendix: 2016 world health organization classification of lymphoid and myeloid Neoplasms 567
Index 571
