Munker / Hiller / Glass Modern Hematology

Biology and Clinical Management
2. Auflage 2007
ISBN: 978-1-59745-149-9
Verlag: Humana Press
Format: PDF
Kopierschutz: 1 - PDF Watermark

E-Book, Englisch, Band 864, 498 Seiten

Reihe: Contemporary Hematology

Now in its second edition, Modern Hematology: Biology and Clinical Management reflects the major advances in the understanding, diagnosis, and treatment of blood disorders. It describes the latest clinical and scientific developments as well as details targeted and molecular therapies. The book brings together facts, concepts, and protocols important for the practice of hematology. In 23 chapters, all major blood diseases are covered, as well as rare diseases that are of scientific interest. As in the previous edition, each chapter is illustrated by tables, figures, and a selection of color plates.

Autoren/Hrsg.

Munker, Reinhold

Hiller, Erhard

Glass, Jonathan

Paquette, Ronald

Inhaltsverzeichnis

1;FOREWORD;6
2;PREFACE;7
3;CONTENTS;8
4;CONTRIBUTORS;10
5;COLOR PLATES;11
6;1 Basic Biology of Hemopoiesis;12
6.1;CONTENTS;12
6.2;1. INTRODUCTION;12
6.3;2. COMPONENTS OF BLOOD AND BONE MARROW;13
6.4;3. ERYTHROPOIESIS;16
6.4.1;3.1. Hemoglobin;17
6.4.2;3.2. Iron Metabolism;18
6.4.3;3.3. The Red Blood Cell;19
6.5;4. MYELOPOIESIS;20
6.5.1;4.1. Eosinophils;21
6.5.2;4.2. Basophils;21
6.5.3;4.3. Mast Cells;21
6.5.4;4.4. Monocytes;22
6.5.5;4.5. Macrophages;22
6.5.6;4.6. Dendritic Cells;22
6.6;5. MEGAKARYOPOIESIS;23
6.7;6. LYMPHATIC TISSUES AND IMMUNE RESPONSE 6.1. Development and Organs of the Lymphoid System;23
6.7.1;6.1. Development and Organs of the Lymphoid System;23
6.7.2;6.2. B-Lymphocytes;24
6.7.3;6.3. T-Lymphocytes;25
6.7.4;6.4. Natural Killer Cells;25
6.8;7. PLASMA COMPONENTS;26
6.9;8. DIAGNOSTIC PROCEDURES IN HEMATOLOGY ;26
6.9.1;8.1. Bone Marrow Aspiration;26
6.9.2;8.2. Bone Marrow Biopsy;28
6.9.3;8.3. Peripheral Blood Film;29
6.10;SUGGESTED READING;29
7;2 Molecular Biology and Cytokines;30
7.1;CONTENTS;30
7.2;1. INTRODUCTION;30
7.3;2. RECOMBINANT DNA TECHNOLOGY;31
7.4;3. CLONALITY OF TUMORS;31
7.4.1;3.1. Cytogenetics;32
7.4.2;3.2. Molecular Cytogenetics;33
7.4.3;3.3. Rearrangement of Immunoglobulin Genes or of the T- Cell Receptor;33
7.4.4;3.4. Light-Chain Restriction;34
7.4.5;3.5. Loss of Heterozygosity;34
7.4.6;3.6. X-Inactivation Assays;34
7.4.7;3.7. Small RNA Molecules in Hematology;35
7.5;4. DIAGNOSTIC APPLICATIONS OF MOLECULAR BIOLOGY AND GENE THERAPY;36
7.5.1;4.1. Viral Vectors and Gene Delivery Systems;37
7.5.2;4.2. Nonviral Vector Systems;38
7.5.3;4.3. Clinical Gene Therapy Protocols;38
7.6;5. CYTOKINES ;40
7.6.1;5.1. Colony- Stimulating Factors;40
7.6.2;5.2. Pegfilgrastim;43
7.6.3;5.3. Thrombopoietin;43
7.6.4;5.4. Erythropoietin;44
7.6.5;5.5. Darbepoietin-;45
7.6.6;5.6. Stem Cell Factor;48
7.6.7;5.7. FLT3 Ligand;48
7.6.8;5.8. Tumor Necrosis Factor-;49
7.6.9;5.9. Fas Ligand;49
7.6.10;5.10. TNF-Related Apoptosis-Inducing Ligand;49
7.6.11;5.11. Transforming Growth Factor-;49
7.6.12;5.12. Interleukins;50
7.6.13;5.13. Interferons;50
7.7;SUGGESTED READING;51
8;3 Supportive Care in Hematology;52
8.1;CONTENTS;52
8.2;1. INTRODUCTION;52
8.3;2. DIAGNOSING SUSPECTED INFECTION IN NEUTROPENIC PATIENTS;53
8.4;3. TREATING INFECTIONS IN NEUTROPENIC PATIENTS;55
8.4.1;3.1. Empirical Treatment;57
8.4.2;3.2. Documented Bacterial Infections;59
8.4.3;3.3. Documented Fungal Infections;60
8.5;4. PROPHYLAXIS OF INFECTIONS IN NEUTROPENIC PATIENTS;65
8.6;5. VENOUS ACCESS;67
8.7;6. FURTHER CONSIDERATIONS;68
8.8;REFERENCES;69
9;4 Transplantation of Stem Cells From Bone Marrow, Peripheral Blood, and the Umbilical Cord;73
9.1;CONTENTS;73
9.2;1. DEFINITIONS AND INTRODUCTION;73
9.3;2. INDICATIONS FOR SCT;74
9.4;3. SELECTION OF A TRANSPLANT TYPE;75
9.5;4. STEM CELL DONOR SELECTION AND EVALUATION;76
9.6;5. UMBILICAL CORD BLOOD STEM CELLS;77
9.7;6. STEM CELL COLLECTION;78
9.8;7. CONDITIONING THERAPY;79
9.9;8. BLOOD PRODUCT ADMINISTRATION;80
9.10;9. PREVENTION AND TREATMENT OF POSTTRANSPLANT INFECTIONS;82
9.11;10. GRAFT FAILURE;84
9.12;11. GRAFT-VS-HOST DISEASE;85
9.13;12. POSTTRANSPLANT PNEUMONIA;88
9.14;13. HEPATIC VENO-OCCLUSIVE DISEASE;90
9.15;14. THROMBOTIC MICROANGIOPATHY;91
9.16;SUGGESTED READING;92
10;5 Anemias;93
10.1;CONTENTS;93
10.2;1. INTRODUCTION;93
10.3;2. ANEMIAS DUE TO ACUTE BLEEDING;94
10.4;3. ANEMIAS DUE TO CHRONIC BLOOD LOSS;94
10.5;4. IRON DEFICIENCY ANEMIA;97
10.5.1;4.1. Pathophysiology;97
10.5.2;4.2. Clinical Signs and Diagnosis;98
10.5.3;4.3. Treatment;99
10.6;5. ANEMIAS OF CHRONIC DISEASES AND OTHER TYPES OF ANEMIAS ;100
10.6.1;5.1. Anemias of Chronic Diseases;100
10.6.2;5.2. Anemias Due to Malnutrition;101
10.6.3;5.3. Anemias Due to Liver Diseases;101
10.6.4;5.4. Anemia of Pregnancy;101
10.6.5;5.5. Anemia of Renal Failure;101
10.6.6;5.6. Anemia of Endocrine Disorders;102
10.6.7;5.7. Thalassemia Minor;102
10.6.8;5.8. Sideroblastic Anemia;102
10.6.9;5.9. Lead Poisoning;103
10.6.10;5.10. Pure Red Cell Aplasia;103
10.7;6. MEGALOBLASTIC ANEMIAS;104
10.7.1;6.1. Physiology of Vitamin B12;104
10.7.2;6.2. Physiology of Folate (Pteroyl Glutamic Acid);105
10.7.3;6.3. Pernicious Anemia;105
10.7.4;6.4. Other Causes of Vitamin B12 Deficiencies;108
10.7.5;6.5. Megaloblastic Anemias Due to Folate Deficiency;108
10.8;SUGGESTED READING;109
11;6 Hemolytic Anemias;110
11.1;CONTENTS;110
11.2;1. INTRODUCTION;110
11.3;2. INHERITED MEMBRANE DEFECTS ;111
11.3.1;2.1. Hereditary Spherocytosis;111
11.3.2;2.2. Hereditary Elliptocytosis;112
11.3.3;2.3. Hereditary Pyropoikilocytosis;112
11.4;3. INHERITED ENZYME DEFECTS;114
11.4.1;3.1. G6PD Deficiency;114
11.4.2;3.2. Pyruvate Kinase Deficiency;115
11.4.3;3.3. Other Red Cell Enzyme Deficiencies Associated With Hemolytic Anemia;116
11.5;4. HEMOGLOBINOPATHIES;116
11.5.1;4.1. Sickle Cell Anemia;116
11.5.2;4.2. Thalassemias;124
11.5.3;4.3. HbC Disease;126
11.5.4;4.4. HbE Syndromes;126
11.5.5;4.5. Methemoglobinemia;126
11.6;5. ACQUIRED HEMOLYTIC ANEMIAS ;127
11.6.1;5.1. Autoimmune Hemolytic Anemias;127
11.6.2;5.2. Paroxysmal Nocturnal Hemoglobinuria;129
11.6.3;5.3. Hemolytic Disease of the Newborn;130
11.6.4;5.4. Special Forms of Acquired Hemolytic Anemias;132
11.7;SUGGESTED READING;135
12;7 Leukocytosis, Leukopenia, and Other Reactive Changes of Myelopoiesis;136
12.1;CONTENTS;136
12.2;1. DEFINITIONS AND PATHOPHYSIOLOGY;136
12.3;2. DIFFERENTIAL DIAGNOSIS OF LEUKOCYTOSIS ( NEUTROPHILIC LEUKOCYTOSIS);137
12.4;3. DIFFERENTIAL DIAGNOSIS OF LEUKOPENIA ( GRANULOCYTOPENIA);139
12.5;4. DRUG-INDUCED NEUTROPENIA (AGRANULOCYTOSIS);140
12.6;5. CYCLIC NEUTROPENIA;142
12.7;6. DIFFERENTIAL DIAGNOSIS OF EOSINOPHILIA;142
12.8;7. DIFFERENTIAL DIAGNOSIS OF BASOPHILIA;143
12.9;8. DIFFERENTIAL DIAGNOSIS OF MONOCYTOSIS;144
12.10;SUGGESTED READING;144
13;8 The Myeloproliferative Syndromes;145
13.1;CONTENTS;145
13.2;1. INTRODUCTION;145
13.3;2. CHRONIC MYELOGENOUS LEUKEMIA;145
13.3.1;2.1. Basic Biology;146
13.3.2;2.2. Clinical Manifestations;147
13.3.3;2.3. Laboratory Abnormalities;147
13.3.4;2.4. Treatment;148
13.4;3. POLYCYTHEMIA VERA;152
13.4.1;3.1. Basic Biology;154
13.4.2;3.2. Clinical Manifestations;154
13.4.3;3.3. Diagnosis;155
13.4.4;3.4. Differential Diagnosis;155
13.4.5;3.5. Treatment;156
13.5;4. ESSENTIAL THROMBOCYTHEMIA;157
13.5.1;4.1. Basic Biology;157
13.5.2;4.2. Clinical Manifestations;158
13.5.3;4.3. Diagnosis;158
13.5.4;4.4. Treatment;158
13.5.5;4.5. Prognosis;159
13.6;5. IDIOPATHIC MYELOFIBROSIS;159
13.6.1;5.1. Basic Biology;159
13.6.2;5.2. Clinical Manifestations;160
13.6.3;5.3. Diagnosis;160
13.6.4;5.4. Differential Diagnosis;160
13.6.5;5.5. Treatment;160
13.7;6. OTHER MYELOPROLIFERATIVE SYNDROMES;161
13.8;SUGGESTED READING;162
14;9 Acute Myelogenous Leukemias;163
14.1;CONTENTS;163
14.2;1. INTRODUCTION;163
14.3;2. ETIOLOGY, BIOLOGY, AND PATHOPHYSIOLOGY OF AML;163
14.4;3. CLINICAL MANIFESTATIONS;166
14.5;4. DIAGNOSIS AND CLASSIFICATION OF AML;167
14.5.1;4.1. Differential Diagnosis of AML;169
14.5.2;4.2. Laboratory Features of AML and Further Laboratory Investigations;169
14.6;5. PROGNOSTIC FACTORS IN AML;171
14.7;6. TREATMENT STRATEGIES ;174
14.7.1;6.1. Induction Treatment;174
14.7.2;6.2. Complications of Treatment;175
14.7.3;6.3. Consolidation Treatment;175
14.7.4;6.4. Role of Allogeneic Bone Marrow Transplantation;176
14.7.5;6.5. Role of Autologous Transplantation;176
14.7.6;6.6. Treatment of Relapsed or Refractory AML;177
14.7.7;6.7. Treatment of APL;177
14.7.8;6.8. Treatment of Secondary Leukemias;178
14.7.9;6.9. Treatment of AML in Older Patients, Antibody Conjugates;178
14.7.10;6.10. New Treatment Strategies and Outlook;179
14.8;SUGGESTED READING;179
15;10 Acute Lymphoblastic Leukemias;181
15.1;CONTENTS;181
15.2;1. INTRODUCTION;181
15.3;2. MOLECULAR BIOLOGY OF ALL;182
15.4;3. CLINICAL MANIFESTATIONS;186
15.5;4. DIAGNOSIS AND CLASSIFICATION OF ALL;186
15.6;5. PROGNOSTIC FACTORS AND COMPLICATIONS IN ALL;191
15.7;6. TREATMENT STRATEGIES;192
15.7.1;6.1. Induction Treatment;192
15.7.2;6.2. Consolidation Treatment;192
15.7.3;6.3. Continuation or Maintenance Treatment;194
15.7.4;6.4. Prophylaxis of Meningeal Leukemia;196
15.7.5;6.5. Supportive Treatment and Prevention of Early Complications;196
15.7.6;6.6. Cure Rates and Late Complications in ALL;197
15.7.7;6.7. Treatment of Relapsed or Refractory ALL and Indications for BMT;198
15.7.8;6.8. Treatment of B-ALL;198
15.7.9;6.9. Treatment of ALL in Older Patients;199
15.7.10;6.10. Use of Monoclonal Antibodies in the Treatment of ALL;199
15.7.11;6.11. Current Treatment Approaches for Ph+ (BCR/ABL+) ALL;199
15.7.12;6.12. Future Perspectives;200
15.8;SUGGESTED READING;200
16;11 Myelodysplastic Syndromes;202
16.1;CONTENTS;202
16.2;1. DEFINITION;202
16.3;2. ETIOLOGY;202
16.4;3. PATHOPHYSIOLOGY;203
16.5;4. CLINICAL AND LABORATORY MANIFESTATIONS;205
16.6;5. PROGNOSIS;207
16.7;6. MDS IN CHILDREN AND YOUNG ADULTS;207
16.8;7. TREATMENT;208
16.9;SUGGESTED READING;212
17;12 Aplastic Anemias;213
17.1;CONTENTS;213
17.2;1. DEFINITION;213
17.3;2. ETIOLOGY;213
17.4;3. PATHOPHYSIOLOGY;215
17.5;4. CLINICAL MANIFESTATIONS;216
17.6;5. DIAGNOSIS AND CLASSIFICATION;216
17.7;6. TREATMENT;219
17.8;7. LATE COMPLICATIONS;221
17.9;SUGGESTED READING;222
18;13 Lymphocytosis, Lymphocytopenia, Lymphadenopathy, and Splenomegaly;223
18.1;CONTENTS;223
18.2;1. INTRODUCTION AND DEFINITIONS;223
18.3;2. DIFFERENTIAL DIAGNOSIS OF A LYMPHOCYTOSIS;224
18.4;3. DIFFERENTIAL DIAGNOSIS OF LYMPHOCYTOPENIA;224
18.5;4. DIFFERENTIAL DIAGNOSIS OF LYMPHADENOPATHY;225
18.6;5. DIFFERENTIAL DIAGNOSIS OF SPLENOMEGALY;227
19;14 Hodgkin’s Lymphoma;230
19.1;CONTENTS;230
19.2;1. INTRODUCTION;230
19.3;2. EPIDEMIOLOGY;231
19.4;3. PATHOGENESIS AND ETIOLOGY;231
19.5;4. CLINICAL FEATURES;232
19.6;5. DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS, STAGING OF HL;232
19.7;6. TREATMENT OPTIONS AND RESULTS 6.1. Treatment;236
19.8;6.2. Prognosis;239
19.9;7. TREATING RELAPSED HL;240
19.10;8. LATE EFFECTS OF TREATMENT;240
19.11;SUGGESTED READING;241
20;15 The Non- Hodgkin’s Lymphomas;242
20.1;CONTENTS;242
20.2;1. INTRODUCTION;242
20.3;2. ETIOLOGY OF NHLs;243
20.4;3. CLASSIFICATION;244
20.5;4. CLINICAL FEATURES AND DIAGNOSTIC STRATEGIES;246
20.6;5. DIFFUSE LARGE B-CELL LYMPHOMA;248
20.6.1;5.1. Prognostic Factors in High-Grade NHL;249
20.6.2;5.2. Gene Expression Profiling in Diffuse Large B-Cell Lymphoma;249
20.6.3;5.3. Treatment;250
20.7;6. FOLLICULAR LYMPHOMA;253
20.7.1;6.1. Clinical Symptoms;253
20.7.2;6.2. Prognosis;254
20.7.3;6.3. Treatment;255
20.7.4;6.4. Novel Strategies;257
20.8;7. MANTLE CELL LYMPHOMA;257
20.9;8. CHRONIC LYMPHOCYTIC LEUKEMIA;258
20.10;9. LYMPHOPLASMACYTOID/-IC LYMPHOMA/ IMMUNOCYTOMA ( INCLUDING WALDENSTRÖM’S DISEASE);263
20.11;10. BURKITT’S LYMPHOMAS, BURKITT-LIKE LYMPHOMAS, AND LYMPHOBLASTIC LYMPHOMAS;264
20.12;11. MYCOSIS FUNGOIDES (CUTANEOUS T-CELL LYMPHOMA) AND SÉZARY SYNDROME;265
20.13;12. SPECIAL FORMS OF NHL ;266
20.13.1;12.1. Hairy Cell Leukemia;266
20.13.2;12.2. Gastric and MALT Lymphomas;269
20.13.3;12.3. Angioimmunoblastic T-Cell Lymphoma;271
20.13.4;12.4. Anaplastic Large-Cell Lymphoma;271
20.13.5;12.5. Lymphomas in Children;272
20.13.6;12.6. Lymphomas in HIV-Infected Individuals;272
20.13.7;12.7. Cerebral Lymphomas;273
20.13.8;12.8. Splenic Marginal Zone Lymphoma;274
20.14;SUGGESTED READING;274
21;16 Multiple Myeloma and Related Paraproteinemias;284
21.1;CONTENTS;284
21.2;1. INTRODUCTION;284
21.3;2. EPIDEMIOLOG Y OF MM;285
21.4;3. PLASMA CELL DYSCRASIAS;285
21.5;4. BIOLOGY OF MM;287
21.6;5. DIAGNOSIS AND STAGING OF MM;290
21.7;6. THERAPY OF MM;297
21.8;7. COMPLICATIONS OF MM;302
21.9;8. AMYLOIDOSIS;306
21.10;SUGGESTED READING;307
22;17 Congenital and Acquired Immunodeficiencies;308
22.1;CONTENTS;308
22.2;1. INTRODUCTION;308
22.3;2. CONGENITAL IMMUNODEFICIENCIES;309
22.3.1;2.1. Severe Combined Immunodeficiencies;315
22.3.2;2.2. Antibody Deficiencies;316
22.3.3;2.3. B-Cell Defects of Unknown Cause;317
22.3.4;2.4. Cellular Deficiencies;318
22.3.5;2.5. Field Defects That Lead to T-Cell Dysfunction;319
22.3.6;2.6. Combined Deficiencies;320
22.3.7;2.7. Defects in Pathways of T-Cell Death and Regulation;320
22.3.8;2.8. Defects of T-Cell Regulation;321
22.3.9;2.9. Defects of DNA Repair;321
22.3.10;2.10. Congenital Defects of Complement Components;322
22.4;3. ACQUIRED OR SECONDARY IMMUNODEFICIENCIES;322
22.5;4. HEMATOLOGICAL ASPECTS OF AIDS;323
22.6;SUGGESTED READING;326
23;18 Infections Relevant to Hematology;327
23.1;CONTENTS;327
23.2;1. INTRODUCTION;327
23.3;2. INFECTIOUS MONONUCLEOSIS AND OTHER EPSTEIN- BARR VIRUS SYNDROMES;328
23.4;3. CMV INFECTIONS;330
23.5;4. PARVOVIRUS B19 INFECTIONS;331
23.6;5. PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY;332
23.7;6. TOXOPLASMOSIS;333
23.8;7. PNEUMOCYSTIS CARINII PNEUMONIAS;334
23.9;8. INFECTIONS CAUSED BY HUMAN HERPESVIRUS-6;335
23.10;9. INFECTIONS CAUSED BY HUMAN HERPESVIRUS-8 ;335
23.10.1;9.1. Introduction;335
23.10.2;9.2. Biology of HHV-8;335
23.10.3;9.3. Diagnosis and Epidemiology of HHV-8 Infections;336
23.10.4;9.4. Treatment of HHV-8-Associated Disorders;337
23.11;SUGGESTED READING;337
24;19 Basic Principles of Hemostasis;338
24.1;CONTENTS;338
24.2;1. NORMAL HEMOSTATIC MECHANISMS;338
24.3;2. ENDOTHELIUM AND THE VASCULAR SYSTEM;339
24.4;3. THE ROLE OF PLATELETS;339
24.5;4. THE ROLE OF BLOOD COAGULATION;342
24.6;5. INHIBITORS OF THE PLASMATIC COAGULATION SYSTEM;345
24.6.1;5.1. Antithrombin;345
24.6.2;5.2. Protein C and Protein S;346
24.7;6. ROLE OF FIBRINOLYSIS;347
24.8;7. CLINICAL EVALUATION OF HEMORRHAGIC DISORDERS;349
24.9;8. LABORATORY EVALUATION OF HEMOSTATIC DISORDERS;350
24.9.1;8.1. Screening Tests;351
24.9.2;8.2. Interpretation the Screening Tests of Hemostasis;352
24.9.3;8.3. Specific Assays of Coagulation;354
24.9.4;8.4. Tests of Hypercoagulability and Fibrinolysis;355
24.10;SUGGESTED READING;356
25;20 Bleeding Disorders;357
25.1;CONTENTS;357
25.2;1. VASCULAR DISORDERS ASSOCIATED WITH BLEEDING;357
25.2.1;1.1. Hereditary Vascular Malformations;357
25.2.2;1.2. Acquired Vascular Disorders;358
25.3;2. PLATELET DISORDERS;360
25.3.1;2.1. Quantitative Disorders of Platelets;360
25.3.2;2.2. Investigation of Thrombocytopenia;360
25.3.3;2.3. Thrombocytopenia Due to Decreased Platelet Production;361
25.3.4;2.4. Thrombocytopenia Due to Increased Platelet Destruction;362
25.3.5;2.5. Immune Thrombocytopenia;362
25.3.6;2.6. Idiopathic Thrombocytopenic Purpura;362
25.3.7;2.7. Treatment in Adults;363
25.3.8;2.8. Other Autoimmune Conditions;365
25.3.9;2.9. Drug-Induced Thrombocytopenia;365
25.3.10;2.10. Posttransfusion Purpura;365
25.3.11;2.11. Neonatal Purpura;366
25.3.12;2.12. Nonimmune Thrombocytopenia;366
25.3.13;2.13. Thrombotic Thrombocytopenic Purpura (Moschcowitz’s Disease) and Hemolytic- Uremic Syndrome ( Gasser’s Syndrome);366
25.3.14;2.14. Cavernous Hemangioma (Kasabach-Meritt Syndrome);368
25.3.15;2.15. Cardiopulmonary Bypass;368
25.3.16;2.16. Hypersplenism;369
25.3.17;2.17. Thrombocytosis;369
25.4;3. QUALITATIVE PLATELET DISORDERS;369
25.4.1;3.1. Bernard-Soulier Syndrome (BSS);370
25.4.2;3.2. Thrombasthenia (Glanzmann Disease);370
25.4.3;3.3. Abnormalities of Platelet Secretion;371
25.4.4;3.4. -Granule Deficiency (Gray Platelet Syndrome, -Storage Pool Disease);371
25.4.5;3.5. Dense Granule Deficiency (;372
25.4.6;3.6. Acquired Disorders of Platelet Function;372
25.5;4. INHERITED DISODERS OF BLOOD COAGULATION;374
25.5.1;4.1. Hemophilias A and B;374
25.5.2;4.2. Von Willebrand Disease;379
25.5.3;4.3. Other Inherited Disorders of Coagulation Factors;381
25.6;5. ACQUIRED COAGULATION DISORDERS;382
25.6.1;5.1. Vitamin K Deficiency;382
25.6.2;5.2. Liver Disease;384
25.6.3;5.3. Disseminated Intravascular Coagulation;384
25.6.4;5.4. Acquired Inhibitors;387
25.6.5;5.5. Massive Transfusions;388
25.7;REFERENCES;389
25.8;SUGGESTED READING;389
26;21 Thrombophilia, Thromboembolic Disease, and Antithrombotic Therapy;391
26.1;CONTENTS;391
26.2;1. THROMBOPHILIA (HYPERCOAGULABILITY);391
26.2.1;1.1. Inherited Hypercoagulable States;392
26.2.2;1.2. Acquired Hypercoagulable States;395
26.2.3;1.3. Management of Thrombophilia;396
26.3;2. THROMBOEMBOLIC DISEASE;396
26.3.1;2.1. Venous Thrombosis;397
26.3.2;2.2. Pulmonary Embolism;399
26.3.3;2.3. Arterial Thrombosis;399
26.4;3. ANTITHROMBOTIC THERAPY;401
26.4.1;3.1. Unfractionated Heparin;402
26.4.2;3.2. Low-Molecular-Weight Heparins;402
26.4.3;3.3. Complications of Heparin Therapy;403
26.4.4;3.4. Oral Anticoagulants;404
26.4.5;3.5. Complications and Interactions;404
26.4.6;3.6. Direct Thrombin Inhibitors (Hirudin);406
26.4.7;3.7. New Antithrombotic Drugs;406
26.4.8;3.8. Fibrinolytic Therapy;407
26.4.9;3.9. Antiplatelet Drugs;409
26.5;REFERENCES;409
26.6;SUGGESTED READING;409
27;22 Transfusion Medicine and Immunohematology;411
27.1;CONTENTS;411
27.2;1. INTRODUCTION;411
27.3;2. RED CELL SEROLOGY ;412
27.3.1;2.1. The ABO, Hh, and Sese Systems;412
27.3.2;2.2. The Rhesus System;413
27.3.3;2.3. Other Red Cell Systems;415
27.3.4;2.4. Diagnostic Methods in Blood Group Serology;416
27.4;3. LEUKOCYTE ANTIGENS;416
27.5;4. BLOOD PRODUCTS AND INDICATIONS FOR BLOOD TRANSFUSION ;421
27.5.1;4.1. Whole Blood;421
27.5.2;4.2. Red Blood Cells;421
27.5.3;4.3. Platelets;422
27.5.4;4.4. Granulocytes;423
27.5.5;4.5. Fresh-Frozen Plasma;424
27.5.6;4.6. Cryoprecipitated Antihemophilic Factor;424
27.5.7;4.7. Immunoglobulins;424
27.6;5. ADVERSE REACTIONS TO BLOOD TRANSFUSION ;425
27.6.1;5.1. Acute Hemolytic Transfusion Reactions;425
27.6.2;5.2. Delayed Hemolytic Transfusion Reaction;427
27.6.3;5.3. Febrile Nonhemolytic Transfusion Reactions;428
27.6.4;5.4. Allergic Reactions;428
27.7;6. DISEASES TRANSMITTED BY BLOOD TRANSFUSION;429
27.7.1;6.1. Bacterial Infections Transmitted by Blood Transfusion;430
27.7.2;6.2. Viral Diseases Transmitted by Blood Transfusion;431
27.7.3;6.3. Parasites Transmitted by Blood Transfusion;438
27.8;7. OTHER ADVERSE EFFECTS OF BLOOD TRANSFUSION ;439
27.8.1;7.1. Iron Overload;439
27.8.2;7.2. Transfusion-Associated GVHD;439
27.8.3;7.3. Transfusion-Related Acute Lung Injury;440
27.9;8. AUTOLOGOUS BLOOD TRANSFUSION;440
27.10;SUGGESTED READING;441
28;23 Storage Disorders in Hematology;443
28.1;CONTENTS;443
28.2;1. GAUCHER DISEASE ;443
28.3;2. HEREDITARY HEMOCHROMATOSIS;445
28.4;3. SECONDARY IRON OVERLOAD;447
28.5;SUGGESTED READING;448
29;Glossary of Cytostatic Drugs and Targeted Therapies;450
30;Laboratory Values;470
31;Program Requirements for Residency Education in Hematology;472
31.1;RECOMMENDATIONS OF THE ACCREDITATION COUNCIL FOR GRADUATE MEDICAL EDUCATION ( ACGME);472
31.2;RECOMMENDATIONS FOR TRAINING BY ASH AND ASCO;473
32;Databases in Hematology;474
32.1;I. TEXTBOOKS OF HEMATOLOGY AND RELATED DISCIPLINES A. MAJOR TEXTBOOKS (> 1000 PAGES);474
32.2;II) PERIODICALS SPECIALIZING IN HEMATOLOGY OR PUBLISHING MAJOR HEMATOLOGIC ARTICLES ( IMPACT FACTOR GIVEN FOR 2004);477
32.3;III. ELECTRONIC DATABASES AND SOURCES OF INFORMATION;477
32.4;IV. SPECIALIZED WEBSITES AND ORGANIZATIONS;478
32.5;V. PROFESSIONAL ASSOCIATIONS OF HEMATOLOGISTS AND RELATED SPECIALISTS OR SCIENTISTS;480
32.6;VI. PATIENT-ORIENTED DATABASES AND SOURCES OF INFORMATION;481
33;Index;482