简 目
Contributors
Preface
PART IClinical Evaluation of the Patient
PARTII TheOrganization of the LymphohematopoieticTissues
PARTIII EpochalHematology
PARTIV Molecular and CellularHematology
PARTV TherapeuticPrinciples
PARTVI The Erythrocyte
PARTVII Neutrophils,Eosinophils,Basophils, and Mast Cells
PARTVIII Monocytes and Macrophages
PARTIX Lymphocytes and Plasma Cells
PARTX MalignantMyeloid Diseases
PARTXI MalignantLymphoid Diseases
PARTXII Hemostasis andThrombosis
PARTXIII Transfusion Medicine
Index
PART I
ClinicalEvaluation of the Patient
1.InitialApproachto the Patient：HistoryandPhysicalExamination
2.Examinationof Blood Cells
3.ExaminationofTheMarrow
4.ConsultativeHematology
PART II
TheOrganization of the LymphohematopoieticTissues
5.Structureof the Marrowand the HematopoieticMicroenvironment
6.The Organization and StructureofLymphoid Tissues
PART III
Epochal Hematology
7.Hematologyof the FetusandNewborn
8.HematologyduringPregnancy
9.Hematology in OlderPersons
PART IV
Molecular and CellularHematology
10.Genetic PrinciplesandMolecular Biology
11.Genomics
12.Epigenetics
13.Cytogeneticsand Genetic Abnormalities
14.MetabolismofHematologicNeoplastic Cells
15.ApoptosisMechanisms：Relevanceto the HematopoieticSystem
16.Cell-CycleRegulationandHematologicDisorders
17.SignalTransductionPathways
18.HematopoieticStem Cells, Progenitors,andCytokines
19.TheInflammatory Response
20.InnateImmunity
21.Dendritic Cells andAdaptiveImmunity
PARTV
TherapeuticPrinciples
22.PharmacologyandToxicityofAntineoplastic Drugs
23.Hematopoietic Cell Transplantation
24.Treatment of InfectionsinTheImmunocompromisedHost
25.AntithromboticTherapy
26.Immune Cell Therapy
27.VaccineTherapy
28.TherapeuticApheresis：Indications,Efficacy,andComplications
29.Gene TherapyforHematologicDiseases
30.RegenerativeMedicine：MultipotentialCellTherapy for
TissueRepair
PARTVI
The Erythrocyte
31.StructureandCompositionof the Erythrocyte
32.Erythropoiesis
33.Erythrocyte Turnover
34.ClinicalManifestations and Classification ofErythrocyteDisorders
35.AplasticAnemia：Acquiredand Inherited.
36. Pure Red Cell Aplasia
37. Anemia of ChronicDisease
38. The Congenital DyserythropoieticAnemias
39. Paroxysmal Nocturnal Hemoglobinuria
40. ParoxysmalNocturnalHemoglobinuria
41. Folate,Cobalamin,andMegaloblasticAnemias
42.IronMetabolism
43.Iron Deficiency andOverload
44. Anemia Resulting fromOtherNutritionalDeficiencies
45. Anemia AssociatedwithMarrowInfiltration
46. Erythrocyte MembraneDisorders
47. Erythrocyte Enzyme Disorders
48. TheThalassemias： Disorders of GlobinSynthesis
49. DisordersofHemoglobinStructure：Sickle CellAnemiaandRelatedAbnormalities
50. Methemoglobinemiaand Other Dyshemoglobinemias
51. FragmentationHemolyticAnemia
52. Erythrocyte Disordersas a ResultofChemicalandPhysicalAgents
53. HemolyticAnemiaResultingfromInfectionswithMicroorganisms
54. HemolyticAnemiaResultingfromImmuneInjury
55. AlloimmuneHemolytic Disease of the FetusandNewborn
56. HypersplenismandHyposplenism
57. Primary and Secondary Erythrocytoses
58.ThePorphyrias
59.PolyclonalandHereditarySideroblasticAnemias
PARTVII
Neutrophils,Eosinophils,Basophils, and Mast Cells
60. StructureandCompositionofNeutrophils,Eosinophils,andBasophils
61. Production,Distribution,andFateofNeutrophils
62. EosinophilsandRelatedDisorders
63. Basophils,Mast Cells, andRelatedDisorders
64. Classificationand Clinical ManifestationsofNeutrophilDisorders
65. NeutropeniaandNeutrophilia
66. DisordersofNeutrophilFunction
PARTVIII
Monocytes and Macrophages
67. Structure,Receptors,andFunctionsofMonocytesandMacrophages
68. Production,Distribution,andActivationofMonocytesandMacrophages
69. Classificationand ClinicalManifestationsof DisordersofMonocytesandMacrophages
70. MonocytosisandMonocytopenia
71. Inflammatory andMalignantHistiocytosis
72. Gaucher Disease andRelatedLysosomalStorageDiseases
73. PART IX
Lymphocytes and Plasma Cells
73.The StructureofLymphocytes andPlasma Cells
74.LymphopoiesisFunctionsof B LymphocytesandPlasma Cells inImmunoglobulinProduction
75. Functionsof T Lymphocytes：T-CellReceptorsforAntigen
76. FunctionsofNaturalKiller Cells
77. Classificationand Clinical ManifestationsofLymphocyteandPlasma Cell Disorders
79.LymphocytosisandLymphocytopenia
80.Immunodeficiency Diseases
81.HematologicManifestationsofAcquiredImmunodeficiencySyndrome
82.MononucleosisSyndromes
PART X
MalignantMyeloid Diseases
83.Classification and ClinicalManifestationsoftheClonalMyeloidDisorders
84.PolycythemiaVera
85.EssentialThrombocythemia
86.Primary Myelofibrosis
87.MyelodysplasticSyndromes
88.AcuteMyelogenousLeukemia
89.ChronicMyelogenousLeukemiaandRelatedDisorders
PART XI
MalignantLymphoid Diseases
90.ClassificationofMalignantLymphoid Disorders
91.AcuteLymphoblasticLeukemia
92.ChronicLymphocyticLeukemia
93.Hairy Cell Leukemia
94.LargeGranularLymphocyticLeukemia
95.General ConsiderationsforLymphomas：Epidemiology,Etiology,Heterogeneity,and Primary Extranodal Disease
96.PathologyofLymphomas.
97.HodgkinLymphoma
98.Diffuse Large B-Cell Lymphoma andRelated Diseases
99.FollicularLymphoma
100.Mantle Cell Lymphoma
101.MarginalZone B-Cell Lymphomas
102.BurkittLymphoma
103.CutaneousT-CellLymphomaMycosisFungoidesandSzarySyndrome
104.MatureT-CellandNaturalKiller Cell Lymphomas
105.Plasma Cell Neoplasms： General Considerations
106.EssentialMonoclonalGammopathy
107.Myeloma.
108.ImmunoglobulinLight-ChainAmyloidosis
109.Macroglobulinemia.
110.Heavy-Chain Disease
PART XII
Hemostasis andThrombosis
111.Megakaryopoiesis andThrombopoiesis
112.PlateletMorphology,Biochemistry,and Function
113.Molecular Biology andBiochemistryof theCoagulationFactorsandPathwaysofHemostasis
114.ControlofCoagulationReactions
115.VascularFunction in Hemostasis
116.Classification, Clinical Manifestations, andEvaluationofDisordersofHemostasis
117.Thrombocytopenia
118.Heparin-Induced Thrombocytopenia
119.ReactiveThrombocytosis
120.HereditaryQualitativePlateletDisorders
121.AcquiredQualitativePlateletDisorders
122.TheVascularPurpuras
123. Hemophilia A andHemophilia B
124. InheritedDeficiencies of Coagulation FactorsII,V,V VIII, VII, X, XI, and XII
125. Hereditary Fibrinogen Abnormalities
126. von Willebrand Disease
127. Antibody-Mediated Coagulation FactorDeficiencies
128. HemostaticAlterations in Liver Disease andLiverTransplantation
129. DisseminatedIntravascularCoagulation
130.HereditaryThrombophilia
131. The AntiphospholipidSyndrome
132. Thrombotic Microangiopathies
133.VenousThrombosis
134. Atherothrombosis： Disease Initiation,Progression,andTreatment
135. Fibrinolysis andThrombolysis
PART XIII
Transfusion Medicine
136. Erythrocyte AntigensandAntibodies
137. Human Leukocyte andPlateletAntigens
138. Blood Procurementand Red Cell Transfusion
139. Preservationand Clinical UseofPlatelets
Index
內容試閱：
前 言PREFACE
The first edition of Williams Hematology n Hematology was publishedin 1972. This, our 9th edition, will represent our continued efforts over nearly one-half century to provide the most current concepts of the pathophysiology and treatment of hematologic diseases.
The rate of growth in our understanding of diseases of blood cells and coagulation pathways provides a challenge for editors of a comprehensive textbook of hematology. The sequencing of individual genomes, analysis of the dark DNA and noncoding RNAs, advances in knowledge in proteomics, metabolomics, and other -omics fields, as applied to hematologic disorders, have accelerated the understanding of the pathogenesis of the diseases of our interest. The rate at which basic knowledge in molecular and cellular biology and immunology has been translated into improved diagnostic and therapeutic methods is equally impressive. Specific molecular targets for therapy in several hematologic disorders have become reality, and it is not hyperbole to state that hematology is the poster child for the rational design of therapeutics applicable to other fields of medicine.
This edition of Williams Hematology includes changes designed tofacilitate ease of access to information, both within the book and its associated links, and has been modestly reorganized to reflect our greater understanding of the origins of hematologic disorders. Each chapter has been revised or rewritten to provide current information. Four new chapters have been added and other notable changes have been made. Chapter 4 Consultative Hematology is new to this edition. The chapter Epigenetics and Genomics has been divided into separate chapters to reflect the growth of knowledge in those disciplines. Chapter 14, Metabolism of Hematologic Neoplastic Cells is new, as this topic has become the basis of multiple potential drug targets for hematologic disease. A section on Autophagy has been added to Chap 15 Apoptosis Mechanisms： Relevance to the Hematopoietic System, as the topic is becoming increasingly important for understanding of the physiology of blood cell development; and an independent chapter Heparin-Induced Thrombocytopenia Chap 118 has been created to reflect both its pathophysiologic and clinical importance. Recognizing that at the heart of diagnostic hematology is blood and marrow cell morphology, we have continued our incorporation of informative color images of the relevant disease topics in each chapter, allowing easy access to illustrations of cell morphology important to diagnosis.
The 9th edition of Williams Hematology is also available online,as part of the excellent www.accessmedicine.com website. With direct links to a comprehensive drug therapy database and to other important medical texts, including Harrisons Principles of Internal Medicine and Goodman and Gilmansof Williams Hematology also includes PubMed links to journal articlescited in the references.
In addition, Williams Manual of Hematology will be revised to reflect the diagnostic and therapeutic advances incorporated in the 9th edition of Williams Hematology. The convenient Manual features the most clinically salient content from the parent text, and is useful in time-restricted clinical situations. The Manual will be available for iPhone and other mobile formats.
The readers of the 9th edition of Williams Hematology will note achanging of some of the guard of our editorial group; Drs. Marcel Levi a member of the 8th edition of Williams Manual of Hematology editorial group, Oliver Press, Linda Burns, and Michael Caligiuri have joined continuing editors Drs. Kenneth Kaushansky, Marshall Lichtman, and Josef Prchal in the 9th edition.
The production of this book required the timely cooperation of 101 contributors for the production of 139 chapters. We are grateful for their work in providing this comprehensive and up-to-date text. Despite the growth of both basic and clinical knowledge and the passion that each of our contributors brings to the topic of their chapter, we have been able to maintain the text in a single volume through scrupulous attention to chapter length.
Each editor has had expert administrative assistance in the management of the manuscripts for which they were primarily responsible. We thank Susan Madden in Salt Lake City, Utah; Nancy Press and Deborah Lemon in Seattle, Washington; and Annie Thompson, Rebecca Posey, and Kimberly Morley in Columbus, Ohio for their very helpful participation in the production of the book. Special thanks go to Susan Daley in Rochester, New York, and Marie Brito in Stony Brook, New York, who were responsible for coordinating the management of 139 chapters, including many new figures and tables, and managing other administrative matters, a challenging task that Ms. Daley and Ms. Brito performed with skill and good humor.
Kenneth Kaushansky
Marshall A. Lichtman Joseph T. Prchal
Marcel Levi
Oliver W. Press
Linda J. Burns
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