Contents
Part 1 LABORA T OR Y HEMA T OL OGY
Contributors vi
Preface xvii
Acknowledgments xix
Chapt er 1 Examination of the Blood and Bone Marrow 1
Kristi J . Smoc k
Chapt er 2 Clinical Flow Cytometry 18
Anna P orwit
Chapt er 3 Cytogenetics 48
Patricia T. Greipp, Jess F . Peterson, Linda B. Baughn, and Min Fang
Chapt er 4 Molecular Diagnosis in Hematology 61
Bing Melody Zhang and J ames L. Zehnder
SECTION 1 HEMA T OPOIESIS 69
Chapt er 5 Origin and Development of Blood Cells 69
Daniel K. Bor g er and Robert T . Means Jr .
SECTION 2 THE ER YTHROCYTE 93
Chapt er 6 The Birth, Life, and Death of Red Blood Cells:
Erythropoiesis, the Mature Red Blood Cell, and Cell Destruction 93
John G. Quigley, Robert T. Means Jr., and Bertil Glader
SECTION 3 GRANUL OCYTES AND MONOCYTES 136
Chapt er 7 Neutrophilic Leukocytes 136
Laur a G. Sc huettpelz and Daniel C. Link
Chapt er 8 The Human Eosinophil 168
Manali Mukherjee , P ar ameswar an Nair , and P aig e Lacy
Chapt er 9 Mast Cells and Basophils: Ontogeny, Characteristics,
and Functional Diversity 196
A. Dean Befus, Marianna Kulka, Kelly M. McNagny, and Judah A. Denburg
Chapt er 10 Monocytes, Macrophages, and Dendritic Cells 208
Matthe w Collin, V enetia Bigle y , and Flor ent Ginhoux
SECTION 4 THE L YMPHOCYTES 233
Chapt er 11 Lymphocytes and Lymphatic Organs 233
Christopher Y . P ark and Matthe w M. Klairmont
Part 2 THE NORMAL HEMA T OL OGIC SY STEM
VOLUME 1Chapter 12 B Lymphocytes 267
Jeffrey J. Bednarski II
Chapter 13 T Lymphocytes 288
Justin C. Boucher, Rawan G. Faramand, and Marco L. Davila
Chapter 14 Natural Killer and Innate Lymphoid Cells 309
Matthew R. Lordo, Todd A. Fehniger, Bethany L. Mundy- Bosse, and Aharon G. Freud
Chapter 15 Major Histocompatibility Complex 322
Qiuheng Jennifer Zhang, Michelle Hickey, Maxim Rosario, Nwe Nwe Soe,
Carrie L. Butler, Rebecca A. Sosa, J. Michael Cecka, and Elaine F . Reed
Chapter 16 Complement System 338
Robert A. Brodsky and Evan M. Braunstein
SECTION 5 HEMOSTASIS 356
Chapter 17 Megakaryocytes 356
William Vainchenker, Najet Debili, and Hana Raslova
Chapter 18 Platelet Structure 391
Elisabeth M. Battinelli and Katie Maurer
Chapter 19 Platelet Function in Hemostasis and Thrombosis 402
David C. Calverley
Chapter 20 Blood Coagulation and Fibrinolysis 427
Stephen J. Everse, Thomas Orfeo, Kathleen E. Brummel- Ziedins, and Kenneth G. Mann
Chapter 21 Endothelium: Angiogenesis and the Regulation of Hemostasis 531
George M. Rodgers
Part 3 TRANSFUSION MEDICINE
Chapter 22 Red Cell, Platelet, and White Cell Antigens 540
Eric A. Gehrie, Heather M. Smetana, and Paul M. Ness
Chapter 23 Transfusion Medicine 558
Mrigender Singh Virk, Suchitra Pandey, and Jennifer Andrews
Part 4 DISORDERS OF RED BLOOD CELLS
SECTION 1 INTRODUCTION 600
Chapter 24 Anemia: General Considerations 600
Robert T. Means Jr., and Bertil Glader
SECTION 2 DISORDERS OF IRON METABOLISM 628
Chapter 25 Iron Deficiency and Related Disorders 628
Elizabeta Nemeth, Marie Hollenhorst, and Lawrence T. Goodnough
Chapter 26 Sideroblastic Anemias 656
Mark D. Fleming
Chapter 27 Hemochromatosis 678
James C. Barton and Charles J. Parker
Chapter 28 Porphyrias 702
Makiko Yasuda, John D. Phillips, and Robert J. DesnickSECTION 3 HEMOLYTIC ANEMIA 726
Chapter 29 Hereditary Spherocytosis, Hereditary Elliptocytosis,
and Other Disorders Associated With Abnormalities
of the Erythrocyte Membrane 726
Patrick G. Gallagher and Bertil Glader
Chapter 30 Hereditary Hemolytic Anemias Due to Red Blood Cell
Enzyme Disorders 749
Rachael F . Grace and Bertil Glader
Chapter 31 Autoimmune Hemolytic Anemia 770
Richard C. Friedberg and Clara Lo
Chapter 32 Paroxysmal Nocturnal Hemoglobinuria 792
Charles J. Parker and Russell E. Ware
Chapter 33 Acquired Nonimmune Hemolytic Disorders 821
Robert T. Means Jr., and Bertil Glader
SECTION 4 HEREDITARY DISORDERS OF HEMOGLOBIN STRUCTURE
AND SYNTHESIS 833
Chapter 34 Sickle Cell Anemia and Other Sickling Syndromes 833
Parul Rai, Jane Silva Hankins, and Jeremie E. Estepp
Chapter 35 Thalassemia Syndromes: Quantitative Disorders of Globin
Chain Synthesis 883
Eugene Khandros and Janet L. Kwiatkowski
Chapter 36 Hemoglobins With Altered Oxygen Affinity, Unstable Hemoglobins,
M- Hemoglobins, and Dyshemoglobinemias 933
Madeleine Verhovsek and Martin H. Steinberg
SECTION 5 OTHER RED CELL DISORDERS 945
Chapter 37 Megaloblastic Anemias: Disorders of Impaired DNA Synthesis 945
Sally P . Stabler
Chapter 38 Inherited Aplastic Anemia Syndromes Germline 974
Nina Weichert- Leahey and Akiko Shimamura
Chapter 39 Acquired Aplastic Anemia 995
Amy E. DeZern and Robert A. Brodsky
Chapter 40 Red Cell Aplasia: Acquired and Congenital Disorders 1008
Anupama Narla, Jeffrey M. Lipton, and Robert T. Means Jr.
Chapter 41 Congenital Dyserythropoietic Anemias 1020
Theodosia A. Kalfa, Bertil Glader, and Gary Kupfer
Chapter 42 Anemia of Inflammation and of Systemic Disorders 1033
Robert T. Means Jr.
Chapter 43 Anemias During Pregnancy and the Postpartum Period 1047
Robert T. Means Jr.
Chapter 44 Anemias Unique to the Fetus and Neonate 1053
Robert D. Christensen and Robin K. Ohls
Chapter 45 Erythrocytosis 1070
Robert T. Means Jr., and Bertil GladerSECTION 1 INTRODUCTION 1080
Chapter 46 Diagnostic Approach to the Bleeding Disorders 1080
George M. Rodgers
SECTION 2 THROMBOCYTOPENIA 1095
Chapter 47 Thrombocytopenia: Pathophysiology and Classification 1095
George M. Rodgers
Chapter 48 Thrombocytopenia Caused by Immunologic Platelet Destruction 1097
David C. Calverley, Christopher McKinney, and Christiane D. Thienelt
Chapter 49 Thrombotic Thrombocytopenic Purpura, Hemolytic- Uremic
Syndrome, and Related Disorders 1123
Han- Mou Tsai
Chapter 50 Miscellaneous Causes of Thrombocytopenia 1153
Archana M. Agarwal, and George M. Rodgers
Chapter 51 Bleeding Disorders Caused by Vascular Abnormalities 1162
George M. Rodgers
Chapter 52 Thrombocytosis and Essential Thrombocythemia 1175
Tsewang Tashi and George M. Rodgers
Chapter 53 Qualitative Disorders of Platelet Function 1182
Emanuela Falcinelli, Loredana Bury, and Paolo Gresele
SECTION 3 COAGULATION DISORDERS 1202
Chapter 54 Inherited Coagulation Disorders 1202
Ming Y. Lim and George M. Rodgers
Chapter 55 Acquired Coagulation Disorders 1241
George M. Rodgers and Ming Y. Lim
SECTION 4 THROMBOSIS 1276
Chapter 56 Thrombosis and Antithrombotic Therapy 1276
Stacy A. Johnson and George M. Rodgers
Part 5 DISORDERS OF HEMOSTASIS AND COAGULATION
Part 6 DISORDERS OF LEUKOCYTES, IMMUNODEFICIENCY, AND THE SPLEEN
Chapter 57 Diagnostic Approach to Tissue Examination and Testing 1321
Aaron C. Shaver and Adam C. Seegmiller
Chapter 58 Neutropenia 1331
Nathalie Javidi- Sharifi and Nancy Berliner
Chapter 59 Qualitative Disorders of Leukocytes 1344
Laura C. Michaelis
Chapter 60 Lysosomal Abnormalities of the Monocyte- Macrophage System:
Gaucher and Niemann- Pick Diseases 1359
Margaret M. McGovern and Robert J. Desnick
Chapter 61 Langerhans Cell Histiocytosis 1364
Amit Rajaram and Michael M. Henry
VOLUME 2Chapter 62 Pathology of Langerhans Cell Histiocytosis and Other
Histiocytic Proliferations 1376
Andrew L. Feldman
Chapter 63 Infectious Mononucleosis and Other Epstein- Barr Virus–Related
Disorders 1382
Laura F . Walsh and Richard F . Ambinder
Chapter 64 Primary Immunodeficiency Diseases 1393
Troy R. Torgerson
Chapter 65 Human Immunodeficiency Virus Infection 1409
Ariela Noy and Roy M. Gulick
Chapter 66 Disorders of the Spleen 1423
William C. Chapman Jr., and William C. Chapman
Chapter 67 Tumors of the Spleen 1437
Daniel A. Arber
Part 7 HEMATOLOGIC MALIGNANCIES
SECTION 1 GENERAL ASPECTS 1444
Chapter 68 Hematopoietic Neoplasms: Principles of Pathologic Diagnosis 1444
Daniel A. Arber
Chapter 69 Principles of Targeted Therapies for Hematologic Malignancies 1454
Nitin Jain, Shilpa Paul, Naveen Pemmaraju, and Varsha Gandhi
Chapter 70 Infectious Complications in Hematologic Malignancies 1468
Markus Plate, Alexander Drelick, and Michael J. Satlin
Chapter 71 Immunotherapy 1494
Trisha R. Berger and Marcela V . Maus
Chapter 72 Gene Therapy for Hematopoietic Stem Cell Disorders 1522
Andre Larochelle
SECTION 2 THE ACUTE LEUKEMIAS 1562
Chapter 73 Molecular Genetics of Acute Leukemia 1562
Ridas Juskevicius, Utpal P . Davé, and Mary Ann Thompson
Chapter 74 Diagnosis and Classification of the Acute Leukemias and
Myelodysplastic Syndromes 1595
Daniel A. Arber and Attilio Orazi
Chapter 75 Acute Lymphoblastic Leukemia in Adults 1608
Ehab Atallah and Kristen O’Dwyer
Chapter 76 Acute Myeloid Leukemia in Adults 1627
Ashkan Emadi and Maria R. Baer
Chapter 77 Acute Lymphoblastic Leukemia in Children 1667
Maureen M. O’Brien, Alix E. Seif, and Stephen P . Hunger
Chapter 78 Acute Myeloid Leukemia in Children 1694
Jennifer L. Kamens, Danielle Shin, and Norman Lacayo
Chapter 79 Acute Promyelocytic Leukemia 1721
Yasmin M. Abaza, Martin S. Tallman, and Jessica K. Altman
Chapter 80 Myelodysplastic Syndromes 1737
Namrata S. Chandhok and Mikkael A. Sekeres
Chapter 81 Clonal Hematopoiesis of Indeterminate Potential
and Myeloid Diseases 1754SECTION 3 MYELOPROLIFERATIVE DISORDERS 1763
Chapter 82 Pathology of the Myeloproliferative Neoplasms 1763
Tracy I. George and Devon S. Chabot- Richards
Chapter 83 Chronic Myeloid Leukemia 1782
Ehab Atallah and Michael W. Deininger
Chapter 84 Polycythemia Vera 1808
Laura C. Michaelis and Robert T. Means Jr.
Chapter 85 Myelofibrosis 1821
Andrew T. Kuykendall, Srdan Verstovsek, Eric Padron, and Rami Komrokji
Chapter 86 Eosinophilic Neoplasms and Hypereosinophilic Syndrome 1836
Jason Gotlib
Chapter 87 Systemic Mastocytosis 1849
Dean D. Metcalfe, Jonathan J. Lyons, and Jason Gotlib
SECTION 4 LYMPHOPROLIFERATIVE DISORDERS 1866
Chapter 88 Diagnosis and Classification of Lymphomas 1866
Pedro Horna, Ji Yuan, and Rebecca L. King
Chapter 89 Molecular Genetic Aspects of Non- Hodgkin Lymphomas 1900
Annette S. Kim, Scott B. Lovitch, and Mark A. Murakami
Chapter 90 Non- Hodgkin Lymphoma in Adults 1942
Tarsheen K. Sethi, Shalin Kothari, Erin Mulvey, Francine Foss, John P . Leonard,
and John P . Greer
Chapter 91 Non- Hodgkin Lymphoma in Children 1991
Nitya Gulati and Lisa Giulino- Roth
Chapter 92 Chronic Lymphocytic Leukemia 2010
Sameer A. Parikh, Saad S. Kenderian, Neil E. Kay, and James B. Johnston
Chapter 93 Hairy Cell Leukemia 2049
James B. Johnston, Graeme R. Quest, and Michael R. Grever
Chapter 94 Cutaneous T- Cell Lymphoma: Mycosis Fungoides
and Sézary Syndrome 2072
John A. Zic, Francine Foss, Eva Niklinska, and Jeff P . Zwerner
Chapter 95 Hodgkin Lymphoma in Adults 2116
Reid W. Merryman and Ann LaCasce
Chapter 96 Hodgkin Lymphoma in Children 2133
Christine Moore Smith and Debra L. Friedman
SECTION 5 PLASMA CELL DYSCRASIAS 2144
Chapter 97 Practical Approach to the Evaluation of Monoclonal
Gammopathies 2144
Taxiarchis Kourelis and Francis K. Buadi
Chapter 98 Molecular Genetic Aspects of Plasma Cell Disorders 2152
Peter Leif Bergsagel, Linda B. Baughn, Dragan Jevremovic, and Yan Asmann
Chapter 99 Monoclonal Gammopathy of Undetermined Significance and
Smoldering Multiple Myeloma 2160
S. Vincent Rajkumar, Robert A. Kyle, and Ronald S. Go
Chapter 100 Multiple Myeloma 2175
Wilson I. Gonsalves, Prashant Kapoor, and Shaji K. Kumar
Chapter 101 Immunoglobulin Light Chain Amyloidosis 2238Part 8 HEMATOPOIETIC CELLULAR THERAPY
Chapter 104 Hematopoietic Cell Transplantation 2297
Rachel B. Salit and Frederick R. Appelbaum
Chapter 105 Hematopoietic Cell Transplantation for Nonmalignant Disorders 2314
Mark C. Walters and Nahal Rose Lalefar
Chapter 106 Hematopoietic Cell Transplantation for Hematologic Malignancies 2335
Bhagirathbhai Dholaria, Jennifer Marvin-Peek, and Bipin N. Savani
Chapter 107 Gr aft- vs- Host Disease and Gr aft- vs- T umor Response 2355
Joseph Pidala, Brian C. Betts, Frederick L. Locke, and Claudio Anasetti
Chapter 108 Late Effects After Hematopoietic Cell Transplantation 2371
Paul A. Carpenter
Index I-1
Chapter 102 Waldenström Macroglobulinemia 2267
Stephen M. Ansell and Rafael Fonseca
Chapter 103 Monoclonal Gammopathies of Clinical Significance 2276
Angela Dispenzieri, David Dingli, and Rahma Warsam