Williams Hematology, 9E (MEDICAL/DENISTRY) PDF
Preview Williams Hematology, 9E (MEDICAL/DENISTRY)
Williams Hematology Kaushansky_FM_pi_xxii.indd 1 9/21/15 4:40 PM Notice Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required. The authors and the publisher of this work have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete, and they disclaim all responsibility for any errors or omissions or for the results obtained from use of the information contained in this work. Readers are encouraged to confirm the information contained herein with other sources. For example and in particular, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications for administration. This recommendation is of particular importance in connection with new or infrequently used drugs. Kaushansky_FM_pi_xxii.indd 2 9/21/15 4:40 PM Williams Hematology Ninth Edition Kenneth Kaushansky, MD, MACP Marshall A. Lichtman, MD Senior Vice President for Health Sciences Professor of Medicine and of Biochemistry and Biophysics Dean, School of Medicine University of Rochester Medical Center SUNY Distinguished Professor Rochester, New York Stony Brook University Stony Brook, New York Marcel Levi, MD, PhD Professor of Medicine Josef T. Prchal, MD Dean, Faculty of Medicine Professor of Medicine, Pathology, and Genetics Academic Medical Center Hematology Division University of Amsterdam University of Utah Amsterdam, The Netherlands Salt Lake City, Utah Department of Pathophysiology Linda J. Burns, MD First Faculty of Medicine Professor of Medicine Charles University in Prague Division of Hematology, Oncology and Transplantation Prague, Czech Republic University of Minnesota Minneapolis, Minnesota Oliver W. Press, MD, PhD Acting Director, Clinical Research Division Michael A. Caligiuri, MD Dr. Penny E. Peterson Memorial Chair for Lymphoma Director, Comprehensive Cancer Center Research CEO, James Cancer Hospital and Solove Research Institute Fred Hutchinson Cancer Research Center Professor of Medicine Professor of Medicine and Bioengineering The Ohio State University University of Washington Columbus, Ohio Seattle, Washington New York Chicago San Francisco Athens London Madrid Mexico City Milan New Delhi Singapore Sydney Toronto Kaushansky_FM_pi_xxii.indd 3 9/21/15 4:40 PM Copyright © 2016, by McGraw-Hill Education. All rights reserved. Except as permitted under the United States Copyright Act of 1976, no part of this publication may be reproduced or distributed in any form or by any means, or stored in a database or retrieval system, without the prior written permission of the publisher, with the exception that the program listings may be entered, stored, and executed in a computer system, but they may not be reproduced for publication. 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THE WORK IS PROVIDED “AS IS.” McGRAW-HILL EDUCATION AND ITS LICENSORS MAKE NO GUARANTEES OR WARRANTIES AS TO THE ACCURACY, ADEQUACY OR COMPLETENESS OF OR RESULTS TO BE OBTAINED FROM USING THE WORK, INCLUDING ANY INFORMATION THAT CAN BE ACCESSED THROUGH THE WORK VIA HYPERLINK OR OTHERWISE, AND EXPRESSLY DISCLAIM ANY WARRANTY, EXPRESS OR IMPLIED, INCLUDING BUT NOT LIMITED TO IMPLIED WARRANTIES OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE. McGraw-Hill Education and its licensors do not warrant or guarantee that the functions contained in the work will meet your requirements or that its operation will be uninterrupted or error free. Neither McGraw-Hill Education nor its licensors shall be liable to you or anyone else for any inaccuracy, error or omission, regardless of cause, in the work or for any damages resulting therefrom. McGraw-Hill Education has no responsibility for the content of any information accessed through the work. Under no circumstances shall McGraw-Hill Education and/or its licensors be liable for any indirect, incidental, special, punitive, consequential or similar damages that result from the use of or inability to use the work, even if any of them has been advised of the possibility of such damages. This limitation of liability shall apply to any claim or cause whatsoever whether such claim or cause arises in contract, tort or otherwise. vv CONTENTS Contributors ............................................... ix 16. Cell-Cycle Regulation and Hematologic Disorders.........213 Yun Dai, Prithviraj Bose, and Steven Grant Preface................................................... xxi 17. Signal Transduction Pathways...........................247 Kenneth Kaushansky PART I 18. Hematopoietic Stem Cells, Progenitors, and Cytokines .....257 Clinical Evaluation of the Patient Kenneth Kaushansky 1. Initial Approach to the Patient: History and Physical 19. The Inflammatory Response ............................279 Examination............................................3 Jeffrey S. Warren and Peter A. Ward Marshall A. Lichtman and Linda J. Burns 20. Innate Immunity......................................293 2. Examination of Blood Cells..............................11 Bruce Beutler Daniel H. Ryan 21. Dendritic Cells and Adaptive Immunity..................307 3. Examination of The Marrow.............................27 Madhav Dhodapkar, Crystal L. Mackall, and Ralph M. Steinman Daniel H. Ryan 4. Consultative Hematology................................41 PART V Rondeep S. Brar and Stanley L. Schrier Therapeutic Principles PART II 22. Pharmacology and Toxicity of Antineoplastic Drugs .......315 Benjamin Izar, Dustin Dzube, James M. Cleary, Constantine The Organization of the Lymphohematopoietic Tissues S. Mitsiades, Paul G. Richardson, Jeffrey A. Barnes, and 5. Structure of the Marrow and the Hematopoietic Bruce A. Chabner Microenvironment .....................................53 23. Hematopoietic Cell Transplantation .....................353 Utpal P. Davé and Mark J. Koury Andrew R. Rezvani, Robert Lowsky, and Robert S. Negrin 6. The Organization and Structure of Lymphoid Tissues .......85 24. Treatment of Infections in The Immunocompromised Aharon G. Freud and Michael A. Caligiuri Host.................................................383 Lisa Beutler and Jennifer Babik PART III 25. Antithrombotic Therapy ...............................393 Epochal Hematology Gregory C. Connolly and Charles W. Francis 7. Hematology of the Fetus and Newborn....................99 26. Immune Cell Therapy..................................409 Carolina Berger and Stanley R. Riddell James Palis and George B. Segel 8. Hematology during Pregnancy..........................119 27. Vaccine Therapy ......................................421 Katayoun Rezvani and Jeffrey J. Molldrem Martha P. Mims 9. Hematology in Older Persons...........................129 28. Therapeutic Apheresis: Indications, Efficacy, and Complications ........................................427 William B. Ershler, Andrew S. Artz, and Bindu Kanapuru Robert Weinstein 29. Gene Therapy for Hematologic Diseases..................437 PART IV Hua Fung and Stanton Gerson Molecular and Cellular Hematology 30. Regenerative Medicine: Multipotential Cell Therapy for 10. Genetic Principles and Molecular Biology ................145 Tissue Repair.........................................447 Lynn B. Jorde Jakub Tolar, Mark J Osborn, Randy Daughters, Anannya Banga, and 11. Genomics............................................155 John Wagner Lukas D. Wartman and Elaine R. Mardis 12. Epigenetics...........................................165 PART VI Bradley R. Cairns The Erythrocyte 13. Cytogenetics and Genetic Abnormalities .................173 31. Structure and Composition of the Erythrocyte ............461 L ucy A. Godley, Madina Sukhanova, Gordana Raca, and Narla Mohandas Michelle M. Le Beau 32. Erythropoiesis ........................................479 14. Metabolism of Hematologic Neoplastic Cells..............191 Josef T. Prchal and Perumal Thiagarajan Zandra E. Walton, Annie L. Hsieh, and Chi V. Dang 33. Erythrocyte Turnover..................................495 15. Apoptosis Mechanisms: Relevance to the Hematopoietic Perumal Thiagarajan and Josef Prchal System...............................................203 34. Clinical Manifestations and Classification of Erythrocyte John C. Reed Disorders ............................................503 Josef T. Prchal Kaushansky_FM_pi_xxii.indd 5 9/21/15 4:40 PM vi Contents 35. Aplastic Anemia: Acquired and Inherited.................513 PART VII George B. Segel and Marshall A. Lichtman Neutrophils, Eosinophils, Basophils, and Mast Cells 36. Pure Red Cell Aplasia..................................539 60. Structure and Composition of Neutrophils, Neal S. Young Eosinophils, and Basophils .............................925 37. Anemia of Chronic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .549 C. Wayne Smith Tomas Ganz 61. Production, Distribution, and Fate of Neutrophils .........939 38. Erythropoietic Effects of Endocrine Disorders.............559 C. Wayne Smith Xylina T. Gregg 62. Eosinophils and Related Disorders.......................947 39. The Congenital Dyserythropoietic Anemias...............563 Andrew J. Wardlaw Achille Iolascon 63. Basophils, Mast Cells, and Related Disorders..............965 40. Paroxysmal Nocturnal Hemoglobinuria ..................571 Stephen J. Galli, Dean D. Metcalfe, Daniel A. Arber, and Ann M. Dvorak Charles J. Parker 64. Classification and Clinical Manifestations of 41. Folate, Cobalamin, and Megaloblastic Anemias............583 Neutrophil Disorders ..................................983 Ralph Green Marshall A. Lichtman 42. Iron Metabolism ......................................617 65. Neutropenia and Neutrophilia ..........................991 Tomas Ganz David C. Dale and Karl Welte 43. Iron Deficiency and Overload...........................627 66. Disorders of Neutrophil Function ......................1005 Tomas Ganz Niels Borregaard 44. Anemia Resulting from Other Nutritional Deficiencies.....651 Ralph Green PART VIII 45. Anemia Associated with Marrow Infiltration..............657 Monocytes and Macrophages Vishnu VB Reddy and Josef T. Prchal 67. Structure, Receptors, and Functions of Monocytes and 46. Erythrocyte Membrane Disorders .......................661 Macrophages ........................................1045 Theresa L Coetzer Steven D. Douglas and Anne G. Douglas 47. Erythrocyte Enzyme Disorders..........................689 68. Production, Distribution, and Activation of Monocytes and Wouter W. van Solinge and Richard van Wijk Macrophages ........................................1075 48. The Thalassemias: Disorders of Globin Synthesis ..........725 Steven D. Douglas and Anne G. Douglas David J. Weatherall 69. Classification and Clinical Manifestations of Disorders 49. Disorders of Hemoglobin Structure: Sickle Cell of Monocytes and Macrophages........................1089 Anemia and Related Abnormalities......................759 Marshall A. Lichtman Kavita Natrajan and Abdullah Kutlar 70. Monocytosis and Monocytopenia ......................1095 50. Methemoglobinemia and Other Dyshemoglobinemias .....789 Marshall A. Lichtman Archana M. Agarwal and Josef T. Prchal 71. Inflammatory and Malignant Histiocytosis ..............1101 51. Fragmentation Hemolytic Anemia.......................801 Kenneth L. McClain and Carl E. Allen Kelty R. Baker and Joel Moake 72. Gaucher Disease and Related Lysosomal Storage Diseases..1121 52. Erythrocyte Disorders as a Result of Chemical Ari Zimran and Deborah Elstein and Physical Agents ...................................809 Paul C. Herrmann PART IX 53. Hemolytic Anemia Resulting from Infections Lymphocytes and Plasma Cells with Microorganisms ..................................815 Marshall A. Lichtman 73. The Structure of Lymphocytes and Plasma Cells ..........1137 54. Hemolytic Anemia Resulting from Immune Injury.........823 Natarajan Muthusamy and Michael A. Caligiuri Charles H. Packman 74. Lymphopoiesis.......................................1149 55. Alloimmune Hemolytic Disease of the Fetus Christopher S. Seet and Gay M. Crooks and Newborn.........................................847 75. Functions of B Lymphocytes and Plasma Cells in Ross M. Fasano, Jeanne E. Hendrickson, and Naomi L. C. Luban Immunoglobulin Production ..........................1159 56. Hypersplenism and Hyposplenism.......................863 Thomas J. Kipps Jaime Caro and Srikanth Nagalla 76. Functions of T Lymphocytes: T-Cell Receptors 57. Primary and Secondary Erythrocytoses ..................871 for Antigen..........................................1175 Fabienne McClanahan and John Gribben Josef T. Prchal 58. The Porphyrias........................................889 77. Functions of Natural Killer Cells .......................1189 Giorgio Trinchieri, Richard W. Childs, and Lewis L. Lanier John D. Phillips and Karl E. Anderson 59. Polyclonal and Hereditary Sideroblastic Anemias..........915 78. Classification and Clinical Manifestations of Lymphocyte and Plasma Cell Disorders .................1195 Prem Ponka and Josef T. Prchal Yvonne A. Efebera and Michael A. Caligiuri Kaushansky_FM_pi_xxii.indd 6 9/21/15 4:40 PM Contents vviiii 79. Lymphocytosis and Lymphocytopenia ..................1199 101. Marginal Zone B-Cell Lymphomas .....................1663 Sumithira Vasu and Michael A. Caligiuri Pier Luigi Zinzani and Alessandro Broccoli 80. Immunodeficiency Diseases ...........................1211 102. Burkitt Lymphoma ...................................1671 Hans D. Ochs and Luigi D. Notarangelo Andrew G. Evans and Jonathan W. Friedberg 81. Hematologic Manifestations of Acquired Immunodeficiency 103. Cutaneous T-Cell Lymphoma (Mycosis Fungoides Syndrome...........................................1239 and Sézary Syndrome) ................................1679 Virginia C. Broudy, Robert D. Harrington Larisa J. Geskin 82. Mononucleosis Syndromes ............................1261 104. Mature T-Cell and Natural Killer Cell Lymphomas........1693 Robert F. Betts Neha Mehta, Alison Moskowitz, and Steven Horwitz 105. Plasma Cell Neoplasms: General Considerations..........1707 PART X Guido Tricot, Siegfried Janz, Kalyan Nadiminti, Erik Wendlandt, and Fenghuang Zhan Malignant Myeloid Diseases 106. Essential Monoclonal Gammopathy ....................1721 83. Classification and Clinical Manifestations of the Marshall A. Lichtman Clonal Myeloid Disorders .............................1275 107. Myeloma............................................1733 Marshall A. Lichtman Elizabeth O’Donnell, Francesca Cottini, Noopur Raje, and 84. Polycythemia Vera ...................................1291 Kenneth Anderson Jaroslav F. Prchal and Josef T. Prchal 108. Immunoglobulin Light-Chain Amyloidosis ..............1773 85. Essential Thrombocythemia ...........................1307 Morie A. Gertz, Taimur Sher, Angela Dispenzieri, and Philip A. Beer and Anthony R. Green Francis K. Buadi 86. Primary Myelofibrosis ................................1319 109. Macroglobulinemia...................................1785 Marshall A. Lichtman and Josef T. Prchal Steven P. Treon, Jorge J. Castillo, Zachary R. Hunter, and 87. Myelodysplastic Syndromes............................1341 Giampaolo Merlini Rafael Bejar and David P. Steensma 110. Heavy-Chain Disease.................................1803 88. Acute Myelogenous Leukemia .........................1373 Dietlind L. Wahner-Roedler and Robert A. Kyle Jane L. Liesveld and Marshall A. Lichtman 89. Chronic Myelogenous Leukemia and Related Disorders ...1437 PART XII Jane L. Liesveld and Marshall A. Lichtman Hemostasis and Thrombosis 111. Megakaryopoiesis and Thrombopoiesis..................1815 PART XI Kenneth Kaushansky Malignant Lymphoid Diseases 112. Platelet Morphology, Biochemistry, and Function.........1829 90. Classification of Malignant Lymphoid Disorders..........1493 Susan S. Smyth, Sidney Whiteheart, Joseph E. Italiano Jr., Paul Bray, and Barry S. Coller Robert A. Baiocchi 113. Molecular Biology and Biochemistry of the 91. Acute Lymphoblastic Leukemia ........................1505 Coagulation Factors and Pathways of Hemostasis.........1915 Richard A. Larson Mettine H. A. Bos, Cornelis van ‘t Veer, and Pieter H. Reitsma 92. Chronic Lymphocytic Leukemia .......................1527 114. Control of Coagulation Reactions ......................1949 Farrukh T. Awan and John C. Byrd Laurent O. Mosnier and John H. Griffin 93. Hairy Cell Leukemia..................................1553 115. Vascular Function in Hemostasis.......................1967 Michael R. Grever and Gerard Lozanski Katherine A. Hajjar, Aaron J. Marcus, and 94. Large Granular Lymphocytic Leukemia .................1563 William Muller Pierluigi Porcu and Aharon G. Freud 116. Classification, Clinical Manifestations, and 95. General Considerations for Lymphomas: Epidemiology, Evaluation of Disorders of Hemostasis ..................1985 Etiology, Heterogeneity, and Primary Extranodal Disease..1569 Marcel Levi, Uri Seligsohn, and Kenneth Kaushansky Oliver W. Press and Marshall A. Lichtman 117. Thrombocytopenia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1993 96. Pathology of Lymphomas..............................1587 Reyhan Diz-Küçükkaya and José A. López Randy D. Gascoyne and Brian F. Skinnider 118. Heparin-Induced Thrombocytopenia ...................2025 97. Hodgkin Lymphoma..................................1603 Adam Cuker and Mortimer Poncz Oliver W. Press 119. Reactive Thrombocytosis..............................2035 98. Diffuse Large B-Cell Lymphoma and Related Diseases.....1625 Kenneth Kaushansky Stephen D. Smith and Oliver W. Press 120. Hereditary Qualitative Platelet Disorders ................2039 99. Follicular Lymphoma.................................1641 A. Koneti Rao and Barry S. Coller Oliver W. Press 121. Acquired Qualitative Platelet Disorders .................2073 100. Mantle Cell Lymphoma ...............................1653 Charles S. Abrams, Sanford J. Shattil, and Joel S. Bennett Martin Dreyling 122. The Vascular Purpuras................................2097 Doru T. Alexandrescu and Marcel Levi Kaushansky_FM_pi_xxii.indd 7 9/21/15 4:40 PM viii Contents 123. Hemophilia A and Hemophilia B.......................2113 133. V enous Thrombosis ..................................2267 Miguel A. Escobar and Nigel S. Key Gary E. Raskob, Russell D. Hull, and Harry R. Buller 124. Inherited Deficiencies of Coagulation Factors II, V, 134. A therothrombosis: Disease Initiation, Progression, V+VIII, VII, X, XI, and XIII...........................2133 and Treatment .......................................2281 Flora Peyvandi and Marzia Menegatti Emile R. Mohler III and Andrew I. Schafer 125. Hereditary Fibrinogen Abnormalities ...................2151 135. F ibrinolysis and Thrombolysis .........................2303 Marguerite Neerman-Arbez and Philippe de Moerloose Katherine A. Hajjar and Jia Ruan 126. von Willebrand Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2163 Jill Johnsen and David Ginsburg PART XIII 127. Antibody-Mediated Coagulation Factor Deficiencies......2183 Transfusion Medicine Sean R. Stowell, John S. (Pete) Lollar, and Shannon L. Meeks 136. Erythrocyte Antigens and Antibodies ...................2329 128. Hemostatic Alterations in Liver Disease and Liver Marion E. Reid and Christine Lomas-Francis Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2191 137. Human Leukocyte and Platelet Antigens ................2353 Frank W.G. Leebeek and Ton Lisman Myra Coppage, David Stroncek, Janice McFarland, and Neil Blumberg 129. Disseminated Intravascular Coagulation ................2199 138. B lood Procurement and Red Cell Transfusion............2365 Marcel Levi and Uri Seligsohn Jeffrey McCullough, Majed A. Refaai, and Claudia S. Cohn 130. Hereditary Thrombophilia.............................2221 139. Preservation and Clinical Use of Platelets................2381 Saskia Middeldorp and Michiel Coppens Terry Gernsheimer and Sherrill Slichter 131. The Antiphospholipid Syndrome .......................2233 Index ...................................................2393 Jacob H. Rand and Lucia Wolgast 132. Thrombotic Microangiopathies ........................2253 J. Evan Sadler Kaushansky_FM_pi_xxii.indd 8 9/21/15 4:40 PM iixx CONTRIBUTORS Charles S. Abrams, MD [121] Jennifer Babik, MD, PhD [24] Professor of Medicine, Pathology and Laboratory Medicine Division of Infectious Diseases Vice Chair for Research & Chief Scientific Officer Department of Medicine Department of Medicine University of California University of Pennsylvania School of Medicine San Francisco, California Philadelphia, Pennsylvania Robert A. Baiocchi, MD, PhD [90] Archana M. Agarwal, MD [50] Associate Professor of Medicine Department of Pathology Division of Hematology University of Utah/ARUP Laboratories Department of Internal Medicine Salt Lake City, Utah The Ohio State University Columbus, Ohio Doru T. Alexandrescu, MD [122] Department of Medicine Kelty R. Baker, MD [51] Division of Dermatology Clinical Assistant Professor University of California, San Diego Baylor College of Medicine VA San Diego Health Care System Houston, Texas San Diego, California Anannya Banga, PhD, [30] Carl E. Allen, MD, PhD [71] Assistant Professor Associate Professor of Pediatrics Department of Genetics Texas Children’s Cancer Center/Hematology Cell Biology, and Development, Stem Cell Institute Baylor College of Medicine University of Minnesota Houston, Texas Minneapolis, Minnesota Karl E. Anderson, MD, FACP [58] Jeffrey A. Barnes [22] Professor, Departments of Preventative Medicine and Community Instructor in Medicine Health, Internal Medicine, and Pharmacology and Toxicology Dana-Farber Cancer Institute University of Texas Medical Branch Harvard Medical School Galveston, Texas Boston, Massachusetts Philip A. Beer, MRCP, FRCPath, PhD [85] Kenneth Anderson, MD [107] Wellcome Trust Sanger Institute Dana-Farber Cancer Institute Wellcome Trust Genome Campus, Hinxton Boston, Massachusetts Cambridge, United Kingdom Daniel A. Arber, MD [63] Rafael Bejar, MD, PhD [87] Ronald F. Dorfman, MBBch, FRCPath Professor in Hematopathology Division of Hematology and Oncology Professor of Pathology Moores Cancer Center Stanford University School of Medicine University of California San Diego Stanford University Medical Center La Jolla, California Stanford, California Joel S. Bennett, MD [121] Andrew S. Artz, MD, MS [9] Professor of Medicine Associate Professor of Medicine Division of Hematology-Oncology University of Chicago University of Pennsylvania School of Medicine Chicago, Illinois Philadelphia, Pennsylvania Farrukh T. Awan, MD [92] Carolina Berger, MD [26] Associate Professor of Internal Medicine Fred Hutchinson Cancer Research Center Division of Hematology Seattle, Washington Department of internal Medicine The Ohio State University Comprehensive Cancer Center Columbus, Ohio Kaushansky_FM_pi_xxii.indd 9 9/21/15 4:40 PM
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