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PART I CLINICAL EVALUATION OF THE PATIENT 3

CHAPTER 1 APPROACH TO THE PATIENT [Ernest Beutler/Marshall A. Lichtman/Barry S. Coller/Thomas J. Kipps/Uri Seligsohn/William J. Williams] 3

CHAPTER 2 EXAMINATION OF THE BLOOD [Daniel H. Ryan] 9

CHAPTER 3 EXAMINATION OF THE MARROW [Daniel H.Ryan] 17

CONTRIBUTORS 19

PREFACE 25

SI UNIT CONVERSION TABLE 27

PART II GENERAL HEMATOLOGY 29

CHAPTER 4 STRUCTURE OF THE MARROW AND THE HEMATOPOLETIC MICROENVIRONMENT [Camille N. Abboud/Marshall A. Lichtman] 29

CHAPTER 5 THE LYMAPOID TISSUES [Thomas J. Kipps] 59

CHAPTER 6 THE INFLAMMATORY RESPONSE [Jeffrey S. Warren/Peter A. Ward] 67

CHAPTER 7 HEMATOLOGY OF THE NEWBORN [George B. Segel/James Palis] 77

CHAPTER 8 HEMATOLOGY IN THE AGED [Marshall A. Lichtman/William J. Williams] 93

PART III MOLECULAR AND CELLULAR HEMATOLOGY 103

CHAPTER 9 GENETIC PRINCIPLES AND MOLECULAR BIOLOGY [Ernest Beutler] 103

CHAPTER 10 CYTOGENETICS AND GENE REARRANGEMENT [Michelle M. LeBeau] 115

CHAPTER 11 APOPTOSIS [Roberta A. Gottlieb] 125

CHAPTER 12 CELL CYCLE REGULATION AND HEMATOLOGICAL DISORDERS [Mathias Schmid/Dennis A. Carson] 131

CHAPTER 13 THE CLUSTER OF DIFFERENTIATION ANTIGENS [Thomas J. Kipps] 141

CHAPTER 14 HEMATOPOIETIC STEM CELLS. PROGENITOR CELLS. AND CYTOKINES [Peter J. Quesenberry/Gerald A. Colvin] 153

CHAPTER 15 IMMUNE ACCESSORY MOLECULES AND SIGNAL TRANSDUCTION [Theodore J. Yun/Edward A. Clark] 175

PART IV THERAPEUTIC PRINCIPLES 185

CHAPTER 16 PHARMACOLOGY AND TOXICITY OF ANTINEOPLASTIC DRUGS [Bruce A. Chabner/Wyndham Wilson/Jeffrey Supko] 185

CHAPTER 17 TREATMENT OF INFECTIONS IN THE IMMUNOCOMPROMISED HOST [Sreven M. Beutler] 201

CHAPTER 18 ALLOGENEIC AND AUTOLOGOUS HEMATOPOIETIC CELL TRANSPLANTATION [Robert S. Negrin/Karl G. Blume] 209

CHAPTER 19 GENE THERAPY[Christopher E. Walsh/Johnson M. Liu/Arthur W. Nienhuis] 249

CHAPTER 20 THE USE OF VENOUS ACCESS DEVICES [McDonald K. Horne,Ⅲ/Donna Jo Mayo/Alison G. Freifeld] 257

CHAPTER 21 PAIN MANAGEMENT [Richard B. Patt/Neil M. Ellison] 261

CHAPTER 22 MORPHOLOGY OF THE ERYTHRON [Brian S. Bull] 271

PART V THE ERYTHROCYTE 271

CHAPTER 23 COMPOSITION OF THE ERYTHROCYTE [Ernest Beutler] 289

CHAPTER 24 IRON METABOLISM [Virgil F. Fairbanks/Ernest Beutler] 295

CHAPTER 25 METABOLIC ASPECTS OF FOLIC ACID AND COBALAMIN [Bernard M. Babior] 305

CHAPTER 26 ENERGY METABOLISM AND MAINTENANCE OF ERYTHROCYTES [Ernest Beutler] 319

CHAPTER 27 THE RED CELL MEMBRNAE [Patrick G Gallagher/Bernard G. Forget] 333

CHAPTER 28 STRUCTURE AND FUNCTION OF HEMOGLOBIN [Helen M. Ranney/Vijay Sharma] 345

CHAPTER 29 PRODUCTION AND DESTRUCTION OF ERYTHROCYTES [Ernest Beutler] 355

CHAPTER 30 CLINICAL MANIFESTATIONS AND CLASSIFICATION OF ERYTHAROCYTE DISORDERS [Allan J. Erslev] 369

CHAPTER 31 APLASTIC ANEMIA [Richard K. Shadduck] 375

CHAPTER 32 PURE RED CELL APLASIA [Allan J. Erslev] 391

CHAPTER 33 ANEMIA OF CHRONIC RENAL FAILURE [Jaime Caro/Allan J. Erslev] 399

CHAPTER 34 ANEMIA OF ENDOCRINE DISORDERS [Allan J. Erslev] 407

CHAPTER 35 THE CONGENITAL DYSERYTHROPOIETIC ANEMIAS [Ernest Beutler] 413

CHAPTER 36 PAROXYSMAL NOCTURNAL HEMOGLOBINURIA [Ernest Beutler] 419

CHAPTER 37 THE MEGALOBLASTIC ANEIAS [Bernard M. Babior] 425

CHAPTER 38 IRON DEFICIENCY [Virgil F. Fairbanks/Ernest Beutler] 447

CHAPTER 40 ANEMIA ASSOCIATED WITH MARROW INFILTRATION [Allan J. Erslev] 447

CHAPTER 39 ANEMIA DUE TO OTHER NUTRITIONAL DEFICIENCIES [Ernest Beutler] 471

CHAPTER 41 ANEMIA OF CHRONIC DISEASE [Allan J. Erslev] 481

CHAPTER 42 DISORDERS OF IRON STORAGE AND TRANSPORT [Virgil F. Fairbanks/David J. Brandhagen] 498

CHAPTER 43 HEREDITARY SPHEROCYTOSIS, ELLIPTOCYTOSIS, AND RELATED DISORDERS [Patrick G. Gallagher/Bernard G.Forget] 503

CHAPTER 44 ACANTHOCYTOSIS, STOMATOCYTOSIS, AND RELATED DISORDERS [Patrick G. Gallagher] 519

CHAPTER 45 GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY AND OTHER RED CELL ENZYME ABNORMALITIES [Ernest Beutler] 527

CHAPTER 46 THE THALASSEMIAS [David J. Weatherall] 547

CHAPTER 47 THE SICKLE CELL DISEASES AND RELATED DISORDERS [Ernest Bertler] 581

CHAPTER 48 HEMOGLOBINOPATHIES ASSOCIATED WITH UNSTABLE HEMOGLOBIN [Ernest Beutler] 607

CHAPTER 49 METHEMOGLOBINEMIA AND OTHER CAUSES OF CYANOSIS [Ernest Beutler] 611

CHAPTER 50 TRAUMATIC CARDIAC HEMOLYTIC ANEMIA [Allan J. Erslev] 619

CHAPTER 51 MICROANGIOPATHIC HEMOLYTIC ANEMIA [Jose Martinez] 623

CHAPTER 52 MARCH HEMOGLOBINURIA, SPORTS ANEMIA, AND SPACE ANEMIA [Allan J. Erslev] 627

CHAPTER 53 HEMOLYTIC ANEMIA DUE TO CHEMICAL AND PHYSICAL AGENTS [Ernest Beutler] 629

CHAPTER 54 HEMOLYTIC ANEMIA DUE TO INFECTIONS WITH MICROORGANISMS [Ernest Beutler] 633

CHAPTER 55 ACQUIRED HEMOLYTIC ANEMIA DUE TO WARM-REACTING AUTOANTIBODIES [Charles H. Packman] 639

CHAPTER 56 CRYOPATHIC HEMOLYTIC SYNDROMES [Charles H. Packman] 649

CHAPTER 57 DRUG-RELATED IMMUNE HEMOLYTIC ANEMIA [Charles H. Packman] 657

CHAPTER 58 ALLOIMMUNE HEMOLYTIC DISEASE OF THE NEWBORN [Jayashree Ramasethu/Naomi L. C. Luban] 665

CHAPTER 59 ACUTE BLOOD LOSS ANEMIA [Robert S. Hillman/Chaim Hershko] 677

CHAPTER 60 HYPERSPLENISM AND HYPOSPLENISM [Allan J. Erslev] 683

CHAPTER 61 POLYCYTHEMIA [Ernest Beutler] 689

CHAPTER 62 THE HEMATOLOGIC ASPECTS OF PORPHYRIA [Shigeru Sassa] 703

CHAPTER 63 HEREDITARY AND ACQUIRED SIDEROBLASTIC ANEMIAS [Ernest Beutler] 721

PART VI NEUTROPHILS, EOSINOPHILS, BASOPHILS AND MAST CELLS 729

CHAPTER 64 MORPHOLOGY OF NEUTROPHILS, EOSIOPHILS, AND BASOPHILS [Dorothy Ford Bainton] 729

CHAPTER 65 COMPOSITION AND METABOLISM OF NEUTROPHILS [Ernest Beutler] 745

CHAPTER 66 PRODUCTION. DISTRIBUTION. AND FATE OF NEUTROPHILS [Bernard M. Babior/David W. Golde] 753

CHAPTER 67 FUNCTIONS OF NEUTROPHILS [James E. Smolen/Laurence A. Boxer] 761

CHAPTER 68 EOSINOPHILS AND THEIR DISORDERS [A. J. Wardlaw/A. Barry Kay] 785

CHAPTER 69 BASOPHILS AND MAST CELLS AND THEIR DISORDERS [Stephen J. Galli, Dean D. Metcalfe,Ann M. Dvorak] 801

CHAPTER 70 CLASSIFICATION AND CLINICAL MANIFESTATIONS OF NEUTROPHIL DISORDERS [Marshall A. Lichtman] 817

CHAPTER 71 NEUTROPENIA AND NEUTROPHILIA [David C. Dale] 823

CHAPTER 72 NEUTROPHIL DISORDERS: QUALITATIVE ABNORMALITIES OF THE NEUTROPHIL [Laurence A. Boxer] 835

PART VII　MONOCYTES AND MACROPHAGES 855

CHAPTER 73 MORPHOLOGY OF MONOCYTES AND MACROPHAGES [Steven D. Douglas/Wen-Zhe Ho] 855

CHAPTER 74 BIOCHEMISTRY AND FUNCTION OF MONOCYTES AND MACROPHAGES [Robert I. Lehrer/Tomas Ganz] 865

CHAPTER 75 PRODUCTION. DISTRIBUTION. AND FATE OF MONOCYTES AND MACROPHAGES [Tomas Ganz/Rober I. Lehrer] 873

CHAPTER 76 CLASSIFICATION AND CLINICAL MANIFESTATIONS OF DISORDERS OF MONOCYTES AND MACROPHAGES [Marshall A. Lichtman] 877

CHAPTER 77 MONOCYTOSIS AND MONOCYTOPENIA [Marshall A. Lichtman] 881

CHAPTER 78 INFLAMMATORY AND MALIGNANT HISTIOCYTOSIS [Marshall A. Lichtman/Camille N. Abboud] 887

CHAPTER 79 LIPID STORAGE DISEASES [Ernest Beutler] 899

CHAPTER 80 MORPHOLOGY OF LYMPHOCYTES AND PLASMA CELLS [Stephen M. Baird] 911

PART VIII LYMPHOCYTES AND PLASMA CELLS 911

CHAPTER 81 COMPOSITION AND BIOCHEMISTRY OF LYMPHOCYTES AND PLASMA CELLS [Thomas J. Kipps/Dennis A. Carson] 919

CHAPTER 82 LYMPHOCYTE ONTOGENY [Tucker W. LeBien/Fred E. Bertrand] 927

CHAPTER 83 FUNCTIONS OF B LYMPHOCYTES AND PLASMA CELLS IN IMMUNOGLOBULIN PRODUCTION [Thomas J. Kipps] 937

CHAPTER 84 FUNCTIONS OF T LYMPHOCYTES:T-CELL RECEPTORS FOR ANTIGEN [Thomas J. Kipps] 949

CHAPTER 85 FUNCTIONS OF NATURAL KILLER CELLS [Giorgio Trinchieri/Lewis L. Lanier] 959

CHAPTER 86 CLASSIFICATION AND CLINICAL MANIFESTATION OF LYMPHOCYTE AND PLASMA CELL DISORDERS [Thomas J. Kipps] 965

CHAPTER 87 LYMPHOCYTOSIS AND LYMPHOCYTOPENIA [Thomas J. Kipps] 969

CHAPTER 88 IMMUNODEFICIENCY DISEASES [Fred S. Rosen] 977

CHAPTER 89 ACQUIRED IMMUNODEFICIENCY SYNDROME(AIDS) [Howard A. Liebman/Timothy P. Cooley/Alexandra M. Levine] 985

CHAPTER 90　MONONUCLEOSIS　SYNDROMES [Kuo-Liang Huang/Robert F. Betts] 1011

PART IX MALIGNANT DISEASES 1017

CHAPTER 91 CLASSIFICATION AND CLINICAL MANIFESTATIONS OF THE CLONAL MYELOID DISORDERS [Marshall A. Lichtman/James K. Brennan] 1017

CHAPTER 92 MYELODYSPLASTIC DISORDERS (INDOLENT CLONAL MYELOID DISEASES AND OLIGOBLASTIC LEUKEMIA)[Marshall A.Lichtman/James K.Brennan] 1029

CHAPTER 93 ACUTE MYELOGENOUS LEUKEMIA [Marshall A. Lichtman/Jane L. Liesveld] 1047

CHAPTER 94 CHRONIC MYELOGENOUS LEUKEMIA AND RELATED DISORDERS [Marshall A. Lichtman/Jane L. Liesveld] 1085

CHAPTER 95 IDIOPATHIC MYELOFIBROSIS(AGNOGENIC MYELOID METAPLASIA) [Marshall A. Lichtman] 1125

CHAPTER 96 CLASSIFICATION OF MALIGNANT LYMPHOID DISORDERS [Thomas J. Kipps] 1137

CHAPTER 97 ACUTE LYMPHOBLASTIC LEUKEMIA [Ching-Hon Piu] 1141

CHAPTER 98 CHRONIC LYMPHOCYTIC LEUKEMIA AND RELAED DISEASES [Thomas J. Kipps] 1163

CHAPTER 99 HAIRY CELL LEUKEMIA [Alan Saven] 1195

CHAPTER 100 LARGE GRANULAR LYMPHOCYTIC LEUKEMIA [Thomas P. Loughran/Marshall E. Kadin] 1203

CHAPTER 101 PATHOLOGY OF MALIGNANT LYMPHOMAS [Peter M. Banks] 1207

CHAPTER 102 HODGKIN LYMPHOMA [Sandra J. Horning] 1215

CHAPTER 103 LYMPHOMAS [Kenneth A. Foon/Richard I. Fisher] 1237

CHAPTER 104 PLASMA CELL NEOPLASMS: GENERAL CONSIDERATIONS [Stephen M. Baird] 1263

CHAPTER 105 ESSENTIAL MONOCLONAL GAMMOPATHY [Marshall A. Lichtman] 1271

CHAPTER 106 PLASMA CELL MYELOMA [Bart Barlogie/John Shaughnessy/Nikhil Munshi/Joshua Epstein] 1279

CHAPTER 107 THE AMYLOIDOSIS [Joel N. Buxbaum/Daniel R. Jacobson] 1305

CHAPTER 108 WALDENSTROM MACROGLOBULINEMIA [Thomas J. Kipps] 1317

CHAPTER 109 HEAVY-CHAIN DISEASES [Joel N. Buxbaum/Alice Alexander] 1327

PART X HEMOSTASIS AND THROMBOSIS 1339

CHAPTER 110 MEGAKARYOPOIESIS AND THROMBOPOIESIS [David J. Kuter] 1339

CHAPTER 111 PLATELET MORPHOLOGY，BIOCHEMISTRY AND FUNCTION [Leslie V. Parise/Susan S. Smyth/Barry S. Coller] 1357

CHAPTER 112 MOLECULAR BIOLOGY AND BIOCHEMISTRY OF THE COAGULATION FACTORS AND PATHWAYS OF HEMOSTASIS [Harold R. Roberts/Dougald M. Monroe Ⅲ/Maureane Hoffman] 1409

CHAPTER 113 CONTROL OF COAGULATION REACTIONS [John H. Griffin] 1435

CHAPTER 114 VASCULAR FUNCTION IN HEMOSTASIS [Katherine A. Hajjar/Naomi L. Esmon/Aaron J. Marcus/William Muller] 1451

CHAPTER 115 CLASSIFICATION, CLINICAL MANIFESTATIONS AND EVALUATION OF DISORDERS OF HEMOSTASIS [Uri Seligsohn/Barry S. Coller] 1471

CHAPTER 116 MOLECULAR MECHANISMS OF FIBRINOLYSIS [Katherine A. Hajjar] 1479

CHAPTER 117 THROMBOCYTOPENIA [James N. George/Mujahid A. Rizvi] 1495

CHAPTER 118 THROMBOCYTOSIS AND ESSENTIAL THROMBOCYTHEMIA [Andrew I. Schafer] 1541

CHAPTER 119 HEREDITARY QUALITATIVE PLATELET DISORDERS [Barry S. Coller/Deborah L. French] 1551

CHAPTER 120 ACQUIRED QUALITATIVE PLATELET DISORDERS DUE TO DISEASES. DRUGS. AND FOODS [Sanford J. Shattil/Charles S. Abrams/Joel S. Bennett] 1583

CHAPTER 121 THE VASCULAR PURPURAS [Paul I. Schneiderman] 1603

CHAPTER 122 INHERITED DEFICIENCIES OF COAGULATION FACTORS Ⅱ.Ⅴ.Ⅶ.Ⅸ.AND XIII AND THE COMBINED DEFICIENCIES OF FACTORS V AND VIII OF THE VIAMIN K-DEPENDENT FACTORS [Uri Seligsohn/Gilbert C. White.Ⅱ] 1617

CHAPTER 123 HEMOPHILIA A AND HEMOPHILIA B [Harold R. Roberts/Maureane Hoffman] 1639

CHAPTER 124 HEREDITARY ABNORMALITIES OF FIBRINOGEN [Michael W. Mosesson] 1659

CHAPTER 125 HEMOSTATIC DYSFUNCTION RELAED TO LIVER DISEASES AND LIVER TRANSPLANTATION [Eberhard F. Mammen] 1673

CHAPTER 126 DISSEMINATED INTRAVASCULAR COAGULATION [Uri Seligsohn] 1677

COLOR PLATES I-XXV(BETWEEN PAGES 1676 AND 1677) 1677

CHAPTER 127 HEREDITARY THROMBOPHILIA [Scott H. Goodnight/John H. Griffin] 1697

CHAPTER 128 THE ANTIPHOSPHOLIPID SYNDROME [Jacob H. Rand] 1715

CHAPTER 129 VENOUS THROMBOSIS [Gary E. Raskob/Russell D. Hull/Graham F. Pineo] 1735

CHAPTER 130 ATHEROSCLEROSIS, THROMBOSIS,AND CORONARY ARTERY DISEASE [Mark B. Taubman] 1743

CHAPTER 131 ANTIPLATELET THERAPY [Harvey J. Weiss] 1763

CHAPTER 132 ORAL ANTICOAGULATION [Sam Schulman] 1777

CHAPTER 133 HEPARIN, HIRUDIN, AND RELATED AGENTS [Gary E. Raskob/Russell D. Hull/Graham F. Pineo] 1793

CHAPTER 134 FIBRINOLYTIC THERAPY [Joseph Loscalzo] 1803

CHAPTER 135 VON WILLEBRAND DISEASE [David Ginsburg] 1813

CHAPTER 136 DISORDERS OF FIBRINOLYSIS AND USE OF ANTIFBRINOLYTIC AGENTS [Frdor Bachmann] 1829

PART XI TRANSFUSION MEDICINE 1843

CHAPTER 137 ERYTHROCYTE ANTIGENS AND ANTIBODIES [Loni Calhoun/Lawrence D. Petz] 1843

CHAPTER 138 HUMAN LEUKOCYTE AND PLATELET ANTIGENS [Karen A. Sullivan/Thomas J. Kipps] 1859

CHAPTER 139 BLOOD PROCUREMENT AND SCREENING [Jeffrey McCullough] 1871

CHAPTER 140 PRESERVATION AND CLINICAL USE OF ERYTHROCYTES AND WHOLE BLOOD[Ernest Beutler] 1879

CHAPTER 141 COLLECTION AND TRANSFUSION OF LEUKOCYTES, DENDRITIC CELLS, AND STEM CELLS [Nicholas A. Bandarenko/Mark E. Brecher] 1893

CHAPTER 142 PRESERVATION AND CLINICAL USE OF PLATELETS [Scott Murphy] 1905

CHAPTER 143 PREPARATION AND CLINICAL USE OF PLASMA AND PLASMA FRACTIONS [Kenneth D. Friedman/Jay E. Menitove] 1917

CHAPTER 144 THERAPEUTIC HEMAPHERESIS: INDICATIONS, EFFICACY, COMPLICATIONS [Bruce C. Mcleod] 1935

INDEX 1943