Color Atlas of Clinical Hematology: Molecular and Cellular Basis of Disease, 5th Edition

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Autore: Hoffbrand, Vyas, Campo, Haferlach, Gomez
Editore: Wiley
Anno: 2019
Pagine: 600
ISBN: 9781119057017
Prezzo: 192,00€
I prezzi indicati possono subire variazioni poiché soggetti all'oscillazione dei cambi delle valute e/o agli aggiornamenti effettuati dagli Editori.

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, BCRABL1+ 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 PCM1JAK2 309

Myelodysplastic/Myeloproliferative Neoplasms With Ring Sideroblasts and Thrombocytosis,312

Juvenile Myelomonocytic Leukemia 314

18 Chronic Lymphocytic Leukemia and Other Mature B and TCell Leukemias 317

Mature B‐Cell Leukemias 317

Mature C-Cell Leukemias 328

19 Small BCell 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 BCell 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 NkCell 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

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