Contents SECTION 1: PHYSIOLOGY OF BLOOD

Transcription

1 Contents SECTION 1: PHYSIOLOGY OF BLOOD Chapter 1: Overview of Physiology of Blood 1 Normal Haematopoiesis 1 Red Blood Cells 6 White Blood Cells 15 Immune System 27 Megakaryopoiesis 32 Normal Haemostasis 33 SECTION 2: DISORDERS OF RED BLOOD CELLS (ANAEMIAS) Chapter 2: Approach to Diagnosis of Anaemias 52 Approach to Diagnosis 53 Chapter 3: Anaemias Due to Impaired Red Cell Production 71 Iron Deficiency Anaemia 71 Normal Iron Metabolism 71 Causes of Iron Deficiency Anaemia 75 Clinical Features 76 Laboratory Features 76 Differential Diagnosis 81 Treatment of Iron Deficiency Anaemia 81 Megaloblastic Anaemias 83 Normal Vitamin B 12 Metabolism 83 Normal Folate Metabolism 86 General Morphological Features of Megaloblastic Anaemia 86 Causes of Megaloblastic Anaemia 89 Aplastic Anaemia and Related Disorders 97 Acquired Aplastic Anaemia 98

2 x Essentials of Haematology Constitutional Aplastic Anaemia 106 Pure Red Cell Aplasia 107 Anaemia of Chronic Disorders 108 Pathogenesis 109 Clinical Features 110 Laboratory Features 110 Differential Diagnosis 111 Treatment 111 Sideroblastic Anaemia 111 Sideroblasts 111 Types and Causes 112 Pathogenesis 112 Anaemia of Chronic Renal Failure 114 Pathogenesis 114 Clinical and Laboratory Features 115 Treatment 115 Anaemia of Liver Disease 115 Myelophthisic Anaemia 116 Congenital Dyserythropoietic Anaemias (CDA) 116 CDA Type I 117 CDA Type II 117 CDA Type III 118 Chapter 4: Anaemias Due to Excessive Red Cell Destruction 121 Hereditary Spherocytosis 121 Aetiopathogenesis 121 Inheritance 122 Clinical Features 122 Laboratory Features 123 Diagnosis of Hereditary Spherocytosis 127 Differential Diagnosis 127 Treatment 128 Hereditary Disorders of Haemoglobin 128 General Features and Approach to Diagnosis 128 The Thalassaemias 141 Sickle-Cell Disorders 171 Disorders of Red Cell Enzymes 185 Glucose-6-Phosphate Dehydrogenase Deficiency 185

3 Contents xi Immune Haemolytic Anaemias 192 Classification 192 Haemolytic Disease of the Newborn 202 Rh Haemolytic Disease of the Newborn 203 ABO Haemolytic Disease of Newborn 209 Paroxysmal Nocturnal Haemoglobinuria 210 Pathogenesis 210 Clinical Features 212 Laboratory Features 212 Treatment 215 Prognosis 215 Mechanical Haemolytic Anaemias 216 Microangiopathic Haemolytic Anaemia 216 March Haemoglobinuria 217 Cardiac Haemolytic Anaemia 217 Haemolytic Anaemia Due to Direct Action of Physical, Chemical, or Infectious Agents 217 Physical Agents 217 Chemical Agents 217 Infectious Agents 217 Hypersplenism 218 Normal Structure and Function of Spleen 218 Causes of Splenomegaly 219 Diagnostic Criteria 219 SECTION 3: DISORDERS OF WHITE BLOOD CELLS Chapter 5: Acute Leukaemias 224 Diagnosis and Classification 224 Acute Lymphoblastic Leukaemia 241 Acute Myeloid Leukaemia 252 Chapter 6: Myelodysplastic Syndromes 267 Pathogenesis 267 Classification of MDS 268 Clinical Features 269 Laboratory Features 269 Differential Diagnosis 273 Prognosis 274 Treatment 274

4 xii Essentials of Haematology Chapter 7: Myeloproliferative Neoplasms 277 Pathogenesis 278 Chronic Myeloid Leukaemia 278 Polycythaemia Vera 289 Primary Myelofibrosis (PMF) 294 Essential Thrombocythaemia 296 Chapter 8: Chronic Lymphoid Leukaemias 299 Chronic Lymphocytic Leukaemia 299 Prolymphocytic Leukaemia 306 Hairy Cell Leukaemia 308 Chapter 9: Plasma Cell Dyscrasias 312 Investigations in Plasma Cell Dyscrasias 312 Multiple Myeloma 318 Waldenström s Macroglobulinaemia 329 Monoclonal Gammopathy of Undetermined Significance 332 Chapter 10: Malignant Lymphomas 335 Hodgkin s Lymphoma 336 Non-Hodgkin s Lymphoma 340 Chapter 11: Quantitative and Qualitative Disorders of Leucocytes 350 Disorders of Granulocytes 351 Neutrophilia 351 Leucoerythroblastic Reaction 353 Leukaemoid Reaction 353 Neutropaenia 354 Eosinophilia 356 Basophilia 357 Disorders of Phagocytic Leucocytes Characterised by Morphologic Changes 357 Disorders of Monocyte Macrophage System 360 Monocytosis 360 Storage Disorders 361 Lymphocytosis 364 Infectious Mononucleosis 365 Immunodeficiency Diseases 369 Classification of Immunodeficiency Diseases 370 Chapter 12: Haematopoietic Stem Cell Transplantation 375 Types of Haematopoietic Stem Cell Transplantation (HSCT) 375 Sources of Haematopoietic Stem Cells 380 Recent Advances in HSCT 380

5 Contents xiii SECTION 4: DISORDERS OF HAEMOSTASIS Chapter 13: Approach to the Diagnosis of Bleeding Disorders 382 Clinical Evaluation 382 Laboratory Evaluation 385 Laboratory Tests 385 Specific Tests 391 Chapter 14: Bleeding Disorders Caused by Abnormalities of Blood Vessels (The Vascular Purpuras) 399 Anaphylactoid Purpura (Henoch-Schönlein Purpura, Allergic Purpura) 400 Infections 400 Scurvy 400 Senile Purpura 400 Purpura Simplex 401 Mechanical Purpura 401 Hereditary Haemorrhagic Telangiectasia (Osler-Weber-Rendu Disease) 401 Chapter 15: Bleeding Disorders Caused by Abnormalities of Platelets 402 Thrombocytopaenia 402 Thrombocytosis 413 Disorders of Platelet Function 414 Chapter 16: Disorders of Coagulation 420 Inherited Disorders of Coagulation 420 Haemophilia A 420 von Willebrand Disease 432 Haemophilia B 438 Inherited Disorders of Fibrinogen 439 Acquired Disorders of Coagulation 440 Vitamin K Deficiency 440 Liver Disease (Cirrhosis of Liver) 441 Disseminated Intravascular Coagulation 442 Acquired Inhibitors of Coagulation (Circulating Anticoagulants) 447 Heparin Therapy 450 Oral Anticoagulants 451 Other Acquired Coagulation Disorders 453 SECTION 5: BLOOD TRANSFUSION Chapter 17: Blood Group Systems 456 ABO System 457 The Rh System 460

6 xiv Essentials of Haematology Chapter 18: Serologic and Microbiologic Techniques 463 Serologic Techniques 463 Microbiologic Techniques 473 Chapter 19: Collection of Donor Blood, Processing and Storage 477 Types of Blood Donors 477 Criteria for Selection of Blood Donors 478 Collection of Donor Blood 482 Processing of Donor Blood 484 Storage of Donor Blood Unit 484 Chapter 20: Whole Blood, Blood Components and Blood Derivatives 486 Whole Blood 487 Blood Components 489 Blood Derivatives 495 Chapter 21: Transfusion of Blood to the Recipient 497 Selection of Donor Blood for Whole Blood or Packed Red Cell Transfusion 498 Selection of Donor Plasma 499 Antibody Screening and Identification 499 Compatibility Test 499 Issue of Donor Blood Unit 499 Transfusion of Blood Unit 500 Chapter 22: Adverse Effects of Transfusion 502 Immediate Complications 503 Delayed Complications 507 Complications Associated with Massive Blood Transfusion 512 Chapter 23: Autologous Transfusion 513 Predeposit Autologous Blood Transfusion 513 Acute Normovolaemic Haemodilution 514 Blood Salvage 515 Chapter 24: Alternatives to Blood Transfusion 516 Haematopoietic Growth Factors (HGFs) 517 Red Cell Substitutes 517 APPENDICES Appendix A: Reference Ranges 519 Appendix B: Selected CD Antigens 522 Appendix C: Critical Values in Haematology 525 Suggested Reading 526 Index 529