Contributors

xi

Preface

xix

Acknowledgments

xxi

Introduction

xxiii

VOLUME 1

PART I

LABORATORY HEMATOLOGY

1

Examination of the Blood and Bone Marrow   Sherrie L. Perkins

1

2

Clinical Flow Cytometry   Frixos Paraskevas

21

3

Cytogenetics Sheila N.J. Sait and Maria R. Baer

50

4

Molecular Biology and Hematology   Rebecca L. Shattuck-Brandt and Stephen J. Brandt

70

PART II

THE NORMAL HEMATOLOGIC SYSTEM

Section 1

Hematopoiesis

5

Origin and Development of Blood Cells   Mark J. Koury, Nadim Mahmud, and Melissa M. Rhodes

79

Section 2

The Erythrocyte

6

Erythropoiesis   Emmanuel N. Dessypris and Stephen T. Sawyer

106

7

The Mature Erythrocyte   Marilyn J. Telen

126

8

Destruction of Erythrocytes   Bertil Glader

156

Section 3

Granulocytes and Monocytes

9

Neutrophilic Leukocytes   Keith M. Skubitz

170

10

The Human Eosinophil   Redwan Moqbel, Solomon O. (Wole) Odemuyiwa, Paige Lacy, and Darryl J. Adamko

214

11

Basophilic Leukocytes: Mast Cells and Basophils   A. Dean Befus and Judah A. Denburg

236

12

Mononuclear Phagocytes   Gregory A. Taylor and J. Brice Weinberg

249

13

Phagocytosis   Frixos Paraskevas

281

Section 4

The Lymphocytes

14

Lymphocytes and Lymphatic Organs   Frixos Paraskevas

300

15

B Lymphocytes   Frixos Paraskevas

326

16

T Lymphocytes and Natural Killer Cells   Frixos Paraskevas

358

17

Effector Mechanisms in Immunity   Frixos Paraskevas

402

Section 5

Hemostasis

18

Megakaryocytes   Kenneth Kaushansky

468

19

Platelet Structure and Function in Hemostasis and Thrombosis   David C. Calverley and Christiane D. Thienelt

490

20

Blood Coagulation and Fibrinolysis   Kathleen Brummel-Ziedins, Thomas Orfeo, Nancy Swords Jenny, Stephen J. Everse, and Kenneth G. Mann

528

21

Endothelium: Angiogenesis and the Regulation of Hemostasis   Paul J. Shami and George M. Rodgers

620

PART III

THERAPEUTIC MODALITIES

22

Red Cell, Platelet, and White Cell Antigens   Kathryn E. Webert, James W. Smith, Donald M. Arnold, Howard H.W. Chan, Nancy M. Heddle, and John G. Kelton

631

23

Transfusion Medicine   Susan A. Galel, Dorothy D. Nguyen, Magali J. Fontaine, Lawrence Tim Goodnough, and Maurene K. Viele

672

24

Hematopoietic Stem Cell Transplantation   Vijayakrishna K. Gadi and Richard A. Nash

722

25

Gene Therapy for Hematologic Disorders   John F. Tisdale, Jay N. Lozier, Stacey A. Goodman, and Cynthia E. Dunbar

749

PART IV

DISORDERS OF RED CELLS

Section 1

Introduction

26

Anemia: General Considerations   Robert T. Means, Jr. and Bertil Glader

779

Section 2

Disorders of Iron Metabolism and Heme Synthesis

27

Iron Deficiency and Related Disorders   Nancy C. Andrews

810

28

Sideroblastic Anemias   Sylvia S. Bottomley

835

29

Hemochromatosis   Corwin Q. Edwards

857

30

Porphyria   Sylvia S. Bottomley

881

Section 3

Hemolytic Anemia

31

Hereditary Spherocytosis, Hereditary Elliptocytosis, and Other Disorders Associated with Abnormalities of the Erythrocyte Membrane   Patrick G. Gallagher and Bertil Glader

911

32

Hereditary Hemolytic Anemias Due to Red Blood Cell Enzyme Disorders   Bertil Glader

933

33

Autoimmune Hemolytic Anemia   Richard C. Friedberg and Vandita P. Johari

956

34

Alloimmune Hemolytic Disease of the Fetus and Newborn   Anne F. Eder and Catherine S. Manno

978

35

Paroxysmal Nocturnal Hemoglobinuria   Charles J. Parker and Russell E. Ware

998

36

Acquired Nonimmune Hemolytic Disorders   Robert T. Means, Jr. and Bertil Glader

1021

Section 4

Hereditary Disorders of Hemoglobin Structure and Synthesis

37

Sickle Cell Anemia and Other Sickling Syndromes   Winfred C. Wang

1038

38

Thalassemias and Related Disorders: Quantitative Disorders of Hemoglobin Synthesis   Caterina Borgna-Pignatti and Renzo Galanello

1083

39

Hemoglobins with Altered Oxygen Affinity, Unstable Hemoglobins, M-Hemoglobins, and Dyshcmoglobinemias   Martin H. Steinberg

1132

Section 5

Other Red Cells Disorders

40

Megaloblastic Anemias: Disorders of Impaired DNA Synthesis   Ralph Carmel

1143

41

Inherited Aplastic Anemia Syndromes   Akiko Shimamura and Blanche P. Alter

1173

42

Acquired Aplastic Anemia   Robert A. Brodsky

1185

43

Red Cell Aplasia   Emmanuel N. Dessypris and Jeffrey M. Lipton

1196

44

Congenital Dyserythropoietic Anemias   Sunitha N. Wickramasinghe and Bertil Glader

1212

45

Anemias Secondary to Chronic Disease and Systemic Disorders   Robert Means, Jr.

1221

46

Anemias During Pregnancy and the Postpartum Period   Robert T. Means, Jr.

1239

47

Anemias Unique to the Newborn Period   Robert D. Christensen and Robin K. Ohls

1247

48

Erythrocytosis   Robert T. Means, Jr.

1261

VOLUME 2

PART V

DISORDERS OF HEMOSTASIS AND COAGULATION

Section 1

Introduction

49

Diagnostic Approach to the Bleeding Disorders   George M. Rodgers and Christopher M. Lehman

1273

Section 2

Thrombocytopenia

50

Thrombocytopenia: Pathophysiology and Classification   George M. Rodgers

1289

51

Thrombocytopenia Caused by Immunologic Platelet Destruction   Christiane D. Thienelt and David C. Calverley

1292

52

Thrombotic Thrombocytopenic Purpura, Hemolytic-Uremic Syndrome, and Related Disorders   Han-Mou Tsai

1314

53

Miscellaneous Causes of Thrombocytopenia    Neeraj Agarwal and George M. Rodgers

1326

Section 3

Other Disorders of Primary Hemostasis

54

Bleeding Disorders Caused by Vascular Abnormalities   Matthew M. Rees and George M. Rodgers

1335

55

Essential Thrombocyttiemia and Thrombocytosis   Ayalew Tefferi

1352

56

Qualitative Disorders of Platelet Function   Thomas J. Kunicki and Diane J. Nugent

1361

Section 4

Coagulation Disorders

57

Inherited Coagulation Disorders   Kenneth D. Friedman and George M. Rodgers

1379

58

Acquired Coagulation Disorders   George M. Rodgers

1425

Section 5

Thrombosis

59

Thrombosis and Antithrombotic Therapy   Steven R. Deitcher and George M. Rodgers

1464

PART VI

NONMALIGNANT DISORDERS OF LEUKOCYTES, THE SPLEEN, AND/OR IMMUNOGLOBINS

60

Diagnostic Approach to Malignant, and Nonmalignant Disorders of the Phagocytic and Immune Systems   Thomas L. McCurley and John P. Greer

1509

61.

Neutropenia   Raymond G. Watts

1527

62

Qualitative Disorders of Leukocytes   Keith M. Skubitz

1548

63

Abnormalities of the Monocyte-Macrophage System: The Lysosoma! Storage Diseases   Margaret M. McGovern and Robert J. Desnick

1565

64

Langerhans Cell Histiocytosis   Suman Malempati and H. Stacy Nicholson

1572

65

Pathology of Langerhans Cell Histiocytosis and Other Histiocytic Proliferations   Lawrence M. Weiss and Karen L. Chang

1582

66

Infectious Mononucleosis and Other Epstein-Barr Virus-Related Disorders   Thomas G. Gross

1589

67

Primary Immunodeficiency Syndromes   Anthony R. Hayward

1612

68

Acquired Immunodeficiency Syndrome   Elaine M. Sloand and Jerome E. Groopman

1623

69

Disorders or the Spleen   Matthew R. Porembka, M.B. Majella Doyle, and William C. Chapman

1637

70

Tumors of the Spleen   Daniel A. Arber

1655

PART VII

HEMATOLOGIC MALIGNANCIES

Section 1

General Aspects

71

Hematopoietic Tumors: Principles of Pathologic Diagnosis   Daniel A. Arber and John B. Cousar

1663

72

Complications of Hematopoietic Neoplasms   Madan H. Jagasia

669

73

Principles and Pharmacology of Chemotherapy   Kenneth R. Hande

1694

74

Immunotherapy   Adetola A. Kassim, Sattva S. Neelapu, Larry W. Kwak, and Luc Van Kaer

1721

75

Supportive Care in Hematologic Malignancies   Madhuri Vusirikala

1747

Section 2

The Acute Leukemias

76

Molecular Genetics of Acute Leukemia   Mary Ann Thompson

1791

77

Diagnosis and Classification of the Acute Leukemias and Myelodysplastic Syndrome   David R. Head

1808

78

Acute Lymphoblastic Leukemia in Adults   Haruka Itakura and Steven E. Coutre

1820

79

Acute Myeloid Leukemia in Adults   Maria R. Baer and John P. Greer

1843

80

Acute Lymphoblastic Leukemia in Children   James A. Whitlock and Paul S. Gaynon

1889

81

Acute Myelogenous Leukemia in Children   Robert J. Arceci and Richard Aplenc

1918

82

Acute Promyelocytic Leukemia   Steven L. Soignet and Peter G. Maslak

1938

83

Myelodysplastic Syndromes   Alan F. List, Avery A. Sandberg, and Donald C. Doll

1956

Section 3

Myeloproliterative Disorders

84

Pathology of the Myeloproliferative Diseases   Tracy I. George

1988

85

Chronic Myeloid Leukemia   Kaaren K. Reichard, Richard S. Larson, and Ian Rabinowitz

2006

86

Polycythcmia Vera   Robert T. Means, Jr.

2031

87

Primary Myelofibrosis   Ayalew Tefferi

2045

88

Systemic Mastocytosis   Alexandra S. Worobec and Dean D. Metcalfe

2054

Section 4

Lymphoproliferative Disorders

89

Diagnosis and Classification of Lymphomas   William R. Macon, Thomas L. McCurley, Paul J. Kurtin, and Ahmet Dogan

2071

90

Molecular Genetic Aspects of Non-Hodgkin Lymphomagenesis   Stephan W. Morris, Andreas Rosenwald, Louis M. Staudt, and Justus Duyster

2103

91

Non-Hodgkin Lymphoma in Adults   John P. Greer and Michael E. Williams

2144

92

Non-Hodgkin Lymphoma in Children   John T. Sandlund and Frederick G. Behm

2195

93

Chronic Lymphocytic Leukemia   James B. Johnston, Matthew Seftel, and Spencer B. Gibson

2214

94

Hairy Cell Leukemia   David Szwajcer and James B. Johnston

2256

95

Cutaneous T-Cell Lymphoma: Mycosis Fungoides and Sézary Syndrome   John A. Zic, Amy C. Musiek, Claudio A. Mosse, and John P. Greer

2276

96

Hodgkin Lymphoma   Richard S. Stein and David S. Morgan

2311

Section 5

Plasma Cell Dyscrasias

97

Practical Aspects of the Clinical Approach to Patients with Monoclonal lmmunoglobulin Disorders   Philip R. Greipp and Rafael Fonseca

2342

98

Monoclonal Gammopathies of Undetermined Significance and Smoldering Multiple Myeloma   Robert A. Kyle

2352

99

Multiple Myeloma   Angela Dispenzieri, Martha Q. Lacy, and Philip R. Greipp

2372

100

lmmunoglobulin Light-Chain Amyloidosis (Primary Amyloidosis)   Morie A. Gertz, Martha Q. Lacy, Angela Dispenzieri, and Suzanne R. Hayman

2439

101

Waldenstrom Macroglobulinemia   Rafael Fonseca and Stephen M. Ansell

2471

102

Cryoglobulincmia and Heavy Chain Disease    Angela Dispenzieri, Morie A. Gertz, and Thomas E. Witzig

2484

APPENDICES

Appendix A

Clusters of Differentiation   Frixos Paraskevas

2497

Appendix B

Normal Blood Values: Selected Health-associated Values for Neonatal, Pediatric, and Adult Populations   Sharon M. Geaghan

2583

Appendix C

Comparative Hematology   Harvey A. Ragan

2595

Index

I-1