Additional Information
Book Details
Abstract
With its highly templated, easy-to-use format and new information throughout, the third edition of Hematopathology, a volume in the Foundations in Diagnostic Pathology series, is an essential text for residents and pathologists. Throughout this practical reference, traditional morphologic diagnostic pathology is supplemented with clinical, immunohistochemical, and molecular genetic information. Fully revised to include recent advances in the field, this affordable resource by Dr. Eric D. Hsi is ideal for study and review as well as everyday clinical practice.
- A highly templated format that includes clinical, imaging, and management/prognostic features; pathologic features; ancillary studies; differential diagnosis; and selected bibliography.
- A focus on the specific features of various entities, including differential diagnoses.
- Clinical features, pathologic features, and key facts summarized in quick-reference boxes for fast retrieval of information.
- Hundreds of full-color photomicrographs and gross photographs depict important pathologic features, enabling you to form a differential diagnosis and compare your findings with actual cases.
- Contributions from internationally recognized pathologists, keeping you up to date with the latest information in the field.
- New information on the pathology of hematological disease and the latest World Health Organization update on classification of hematologic malignancies.
- New chapter on the essentials of hemostasis.
- Completely revised chapter on platelets
Table of Contents
Section Title | Page | Action | Price |
---|---|---|---|
Front Cover | cover | ||
Inside Front Cover | ifc1 | ||
Half title page | i | ||
Other books in this series | ii | ||
Hematopathology | iii | ||
Copyright Page | iv | ||
Dedication | v | ||
■ Contributors | vii | ||
■ Foreword | xi | ||
■ Preface | xiii | ||
Table Of Contents | xv | ||
1 Non-Neoplastic Disorders | 1 | ||
1 Red Blood Cell/Hemoglobin Disorders*We gratefully acknowledge the contribution of Steven H. Kroft, MD, who co-authored this chapter in previous editions. | 3 | ||
■ Introduction | 3 | ||
■ Anemias of Blood Loss | 3 | ||
■ Anemias of Decreased Red Blood Cell Production | 3 | ||
Ineffective Erythropoiesis | 3 | ||
Megaloblastic Anemia | 3 | ||
Clinical Features | 5 | ||
Pathologic Features | 5 | ||
Ancillary Studies | 8 | ||
Differential Diagnosis | 11 | ||
Prognosis and Therapy | 11 | ||
Iron Deficiency Anemia | 12 | ||
Clinical Features | 12 | ||
Pathologic Features | 12 | ||
Ancillary Studies | 13 | ||
Differential Diagnosis | 14 | ||
Prognosis and Therapy | 14 | ||
Sideroblastic Anemia | 14 | ||
Clinical Features | 15 | ||
Pathologic Features | 15 | ||
Ancillary Studies | 15 | ||
Differential Diagnosis | 15 | ||
Prognosis and Therapy | 15 | ||
Congenital Dyserythropoietic Anemia | 16 | ||
Clinical Features | 17 | ||
Pathologic Features | 17 | ||
Ancillary Studies | 18 | ||
Differential Diagnosis | 18 | ||
Therapy | 19 | ||
Decreased Effective Erythropoiesis | 19 | ||
Anemia of Chronic Kidney Disease | 19 | ||
Clinical Features | 19 | ||
Pathologic Features | 20 | ||
Ancillary Studies | 20 | ||
Differential Diagnosis | 21 | ||
Prognosis and Therapy | 21 | ||
Anemia of Chronic Disease (Anemia of Inflammation) | 21 | ||
Clinical Features | 21 | ||
Pathologic Features | 22 | ||
Ancillary Studies | 22 | ||
Differential Diagnosis | 23 | ||
Prognosis and Therapy | 23 | ||
Hemolytic Anemias | 23 | ||
Intrinsic Red Cell Defects | 24 | ||
Hemoglobin Disorders | 24 | ||
Structural | 24 | ||
Sickle Cell Disease | 24 | ||
Clinical Features | 24 | ||
Pathologic Features | 25 | ||
Ancillary Studies | 25 | ||
Differential Diagnosis | 29 | ||
Prognosis and Therapy | 29 | ||
Hemoglobin CC Disease | 30 | ||
Clinical Features | 30 | ||
Pathologic Features | 30 | ||
Ancillary Studies | 31 | ||
Differential Diagnosis | 31 | ||
Prognosis and Therapy | 32 | ||
Thalassemias | 32 | ||
Clinical Features | 32 | ||
Radiologic Features | 33 | ||
Pathologic Features | 33 | ||
Ancillary Studies | 33 | ||
Hemoglobin Analysis | 33 | ||
Other Studies | 35 | ||
Differential Diagnosis | 35 | ||
Prognosis and Therapy | 36 | ||
Membrane Disorders | 36 | ||
Hereditary Spherocytosis | 36 | ||
Clinical Features | 37 | ||
Pathologic Features | 37 | ||
Ancillary Studies | 37 | ||
Osmotic Fragility Test | 37 | ||
Other Studies | 38 | ||
Differential Diagnosis | 38 | ||
Prognosis and Therapy | 38 | ||
Hereditary Elliptocytosis and Hereditary Pyropoikilocytosis | 38 | ||
Clinical Features | 39 | ||
Pathologic Features | 39 | ||
Ancillary Studies | 39 | ||
Differential Diagnosis | 41 | ||
Prognosis and Therapy | 41 | ||
Enzyme Disorders | 41 | ||
Glucose-6-Phosphate Dehydrogenase Deficiency | 41 | ||
Clinical Features | 41 | ||
Pathologic Features | 42 | ||
Ancillary Studies | 42 | ||
Differential Diagnosis | 44 | ||
Prognosis and Therapy | 44 | ||
Extrinsic Defects | 44 | ||
Autoimmune Hemolytic Anemia | 44 | ||
Clinical Features | 45 | ||
Pathologic Features | 45 | ||
Ancillary Studies | 47 | ||
Differential Diagnosis | 47 | ||
Prognosis and Therapy | 47 | ||
Microangiopathic Hemolytic Anemia | 47 | ||
Clinical Features | 48 | ||
Pathologic Features | 48 | ||
Ancillary Studies | 48 | ||
Differential Diagnosis | 50 | ||
Prognosis and Therapy | 50 | ||
Parasitic Hemolysis | 51 | ||
Clinical Features | 51 | ||
Pathologic Features | 51 | ||
Ancillary Studies | 53 | ||
Differential Diagnosis | 56 | ||
Prognosis and Therapy | 56 | ||
■ Erythrocytosis | 56 | ||
Suggested Reading | 56.e1 | ||
Megaloblastic Anemia | 56.e1 | ||
Iron Deficiency Anemia | 56.e1 | ||
Sideroblastic Anemia | 56.e1 | ||
Congenital Dyserythropoietic Anemia | 56.e1 | ||
Anemia of Chronic Kidney Disease | 56.e1 | ||
Anemia of Chronic Disease | 56.e1 | ||
Sickle Cell Disease | 56.e1 | ||
Hemoglobin CC Disease | 56.e1 | ||
Thalassemias | 56.e1 | ||
Hereditary Spherocytosis | 56.e1 | ||
Hereditary Elliptocytosis and Pyropoikilocytosis | 56.e2 | ||
Glucose-6-Phosphate Dehydrogenase Deficiency | 56.e2 | ||
Autoimmune Hemolytic Anemia | 56.e2 | ||
Microangiopathic Hemolytic Anemia | 56.e2 | ||
Parasitic Hemolysis | 56.e2 | ||
Erythrocytosis | 56.e2 | ||
2 Hemostasis and Thrombosis | 57 | ||
■ Basic Physiology of Hemostasis and Fibrinolysis | 57 | ||
Coagulation Cascade | 57 | ||
Cell-Based Model of Coagulation | 58 | ||
Physiologic Inhibition of Coagulation | 59 | ||
Fibrinolytic System | 60 | ||
Platelets | 60 | ||
■ Laboratory Evaluation in Hemostatic and Thrombotic Disorders | 62 | ||
Commonly Used Laboratory Assays Related to Hemostatic Disorders | 62 | ||
Laboratory Assays Related to Thrombotic Disorders | 64 | ||
Effect of Anticoagulants on Coagulation Assays | 65 | ||
Evaluation of Platelet Function | 66 | ||
■ Bleeding Disorders | 70 | ||
Hemophilia A and B | 70 | ||
Clinical Features | 70 | ||
Pathologic Features and Test Results | 70 | ||
Differential Diagnosis | 71 | ||
Prognosis and Therapy | 71 | ||
Acquired Hemophilia/Specific Factor Inhibitors | 72 | ||
Clinical Features | 72 | ||
Pathologic Features and Test Results | 72 | ||
Differential Diagnosis | 72 | ||
Prognosis and Therapy | 72 | ||
Other Factor Deficiencies | 73 | ||
Von Willebrand Disease | 73 | ||
Clinical Features | 73 | ||
Pathologic Features and Test Results | 75 | ||
Differential Diagnosis | 76 | ||
Prognosis and Therapy | 77 | ||
Disseminated Intravascular Coagulation | 77 | ||
Clinical Features | 77 | ||
Pathologic Features and Test Results | 77 | ||
Differential Diagnosis | 79 | ||
Prognosis and Therapy | 79 | ||
Fibrinolytic Disorders | 79 | ||
Clinical Features | 79 | ||
Pathologic Features and Test Results | 79 | ||
Differential Diagnosis | 80 | ||
Prognosis and Therapy | 80 | ||
■ Thrombophilic Disorders | 80 | ||
Antiphospholipid Syndrome | 80 | ||
Clinical Features | 80 | ||
Pathologic Features and Laboratory Studies | 81 | ||
Lupus Anticoagulant Testing | 82 | ||
Anticardiolipin Antibody and β2 Glycoprotein 1 Antibody Testing | 82 | ||
Differential Diagnosis | 82 | ||
Prognosis and Therapy | 83 | ||
Natural Anticoagulant Deficiency | 83 | ||
Antithrombin Deficiency | 83 | ||
Clinical Features | 83 | ||
Pathologic Features and Laboratory Studies | 83 | ||
Differential Diagnosis | 84 | ||
Prognosis and Therapy | 84 | ||
Protein C Deficiency | 84 | ||
Clinical Features | 84 | ||
Pathologic Features and Laboratory Studies | 84 | ||
Differential Diagnosis | 85 | ||
Prognosis and Therapy | 85 | ||
Protein S Deficiency | 85 | ||
Clinical Features | 85 | ||
Pathologic Features and Laboratory Studies | 86 | ||
Differential Diagnosis | 86 | ||
Prognosis and Therapy | 86 | ||
Activated Protein C Resistance and the Factor V Leiden Mutation | 86 | ||
Clinical Features | 86 | ||
Pathologic Features and Laboratory Studies | 87 | ||
Differential Diagnosis | 87 | ||
Prognosis and Therapy | 88 | ||
Prothrombin Gene G20210A Mutation | 88 | ||
Clinical Features | 88 | ||
Pathologic Features and Laboratory Studies | 88 | ||
Prognosis and Therapy | 89 | ||
Dysfibrinogenemia | 89 | ||
Clinical Features | 89 | ||
Pathologic Features and Laboratory Studies | 89 | ||
Differential Diagnosis | 90 | ||
Prognosis and Therapy | 90 | ||
Thrombotic Thrombocytopenic Purpura | 90 | ||
Clinical Features | 90 | ||
Pathologic Features and Laboratory Studies | 90 | ||
Differential Diagnosis | 92 | ||
Prognosis and Therapy | 92 | ||
■ Platelet Disorders | 93 | ||
Defects of Platelet Production | 93 | ||
GATA-1–Related Diseases | 93 | ||
Paris-Trousseau Syndrome (FLI1) | 93 | ||
Familial Thrombocytopenia– Leukemia (RUNX1) | 93 | ||
Congenital Amegakaryocytic Thrombocytopenia (CAMT) (MPL) | 93 | ||
Congenital Amegakaryocytic Thrombocytopenia with Radioulnar Synostosis (CTRUS) (HOXA11) | 93 | ||
Thrombocytopenia with Absent Radii (TAR) (RBM8A) | 93 | ||
Defects of Platelet Surface Receptors | 94 | ||
Glanzmann Thrombasthenia | 94 | ||
Clinical Features | 94 | ||
Pathologic Features and Laboratory Studies | 94 | ||
Differential Diagnosis | 94 | ||
Prognosis and Therapy | 96 | ||
Bernard-Soulier Syndrome | 96 | ||
Clinical Features | 96 | ||
Pathologic Features and Laboratory Studies | 97 | ||
Differential Diagnosis | 98 | ||
Prognosis and Therapy | 98 | ||
Platelet-Type von Willebrand Disease | 98 | ||
Glycoprotein IV Disorders | 98 | ||
Collagen Receptor Disorders | 99 | ||
Glycoproteins Ia/IIa | 99 | ||
Glycoprotein VI | 99 | ||
Adenosine Diphosphate Receptor Abnormalities | 99 | ||
Other Surface Glycoprotein Defects | 99 | ||
Platelet Procoagulant Disorders | 99 | ||
Defects of Secretion and Organelle Biogenesis | 99 | ||
Gray Platelet Syndrome | 99 | ||
Clinical Features | 99 | ||
Pathologic Features and Laboratory Studies | 99 | ||
Differential Diagnosis | 100 | ||
Quebec Platelet Syndrome | 100 | ||
Dense Granule Storage Pool Disorders | 100 | ||
Chediak-Higashi Syndrome | 100 | ||
Hermansky-Pudlak Syndrome | 101 | ||
Signal Transduction Disorders | 101 | ||
Cytoskeletal Disorders | 101 | ||
MYH9 Disorders | 101 | ||
Clinical Features | 101 | ||
Pathologic Features and Laboratory Studies | 101 | ||
Differential Diagnosis | 101 | ||
Prognosis and Therapy | 102 | ||
Wiskott-Aldrich Syndrome and X-Linked Thrombocytopenia | 102 | ||
Clinical Features | 102 | ||
Pathologic Features and Laboratory Studies | 103 | ||
Differential Diagnosis | 103 | ||
Acquired Platelet Disorders | 103 | ||
Platelet Disorders With Thrombocytosis | 103 | ||
Acquired Thrombocytopenias With Decreased Platelet Production | 103 | ||
Acquired Thrombocytopenias with Increased Platelet Turnover | 103 | ||
Immune Thrombocytopenic Purpura | 104 | ||
Clinical Features | 104 | ||
Pathologic Features and Laboratory Studies | 104 | ||
Differential Diagnosis | 104 | ||
Prognosis and Therapy | 104 | ||
Alloimmune Thrombocytopenias | 104 | ||
Drug-Induced Thrombocytopenias | 105 | ||
Other Causes of Thrombocytopenia With Increased Platelet Turnover | 105 | ||
Platelet Dysfunction Associated With Other Illnesses | 105 | ||
Summary | 105 | ||
Suggested Readings | 105.e1 | ||
Basic Physiology of Hemostasis and Fibrinolysis | 105.e1 | ||
Platelet Production and Hemostatic Function | 105.e1 | ||
Laboratory Assays Related to Hemostatic Disorders | 105.e1 | ||
Laboratory Assays Related to Thrombotic Disorders | 105.e1 | ||
Effect of Anticoagulants on Coagulation Assays | 105.e1 | ||
Evaluation of Platelet Function | 105.e1 | ||
Bleeding Disorders | 105.e2 | ||
Von Willebrand Disease and DIC | 105.e2 | ||
Fibrinolytic Disorders | 105.e2 | ||
Thrombophilia | 105.e2 | ||
Natural Anticoagulant Deficiency | 105.e3 | ||
Protein C Deficiency | 105.e3 | ||
Protein S Deficiency | 105.e3 | ||
Activated Protein C Resistance and the Factor V Leiden Mutation | 105.e3 | ||
Prothrombin Gene G20210A Mutation | 105.e3 | ||
Dysfibrinogenemia | 105.e3 | ||
Thrombotic Thrombocytopenic Purpura | 105.e3 | ||
Platelet Disorders | 105.e4 | ||
Defects of Platelet Production | 105.e4 | ||
Defects of Platelet Surface Receptors | 105.e4 | ||
Secretion and Organelle Biogenesis | 105.e4 | ||
Cytoskeletal Abnormalities | 105.e4 | ||
Acquired Platelet Disorders | 105.e4 | ||
3 Non-Neoplastic Morphologic Abnormalities of White Blood Cells and Macrophages | 106 | ||
■ Introduction | 106 | ||
Congenital Morphologic Abnormalities of White Blood Cells | 106 | ||
Pelger-Huet Anomaly | 106 | ||
Myelokathexis | 108 | ||
Chédiak-Higashi Syndrome | 108 | ||
Alder-Reilly Anomaly | 109 | ||
May-Hegglin Anomaly | 110 | ||
Vacuolated Lymphocytes | 111 | ||
Acquired Abnormalities of White Blood Cells | 111 | ||
“Toxic” Changes in Leukocytes | 111 | ||
Cytokine Effects | 113 | ||
Megaloblastic and Dysplastic Changes | 113 | ||
Eosinophils | 114 | ||
Reactive Lymphocytes and Lymphocytosis | 114 | ||
Smudge Cells | 114 | ||
Viral Lymphocytosis | 114 | ||
Other Reactive Lymphocyte Changes | 115 | ||
Morphologic Abnormalities in Bone Marrow Macrophages | 115 | ||
Suggested Reading | 117.e1 | ||
Congenital Morphologic Abnormalities of WBCs | 117.e1 | ||
Acquired Morphologic Abnormalities of WBCs | 117.e1 | ||
Morphologic Abnormalities in Macrophages (Bone Marrow) | 117.e2 | ||
4 Reactive Lymph Nodes and Castleman Disease | 118 | ||
■ Normal Lymph Node Anatomy | 118 | ||
■ Viral Infections | 118 | ||
Epstein-Barr Virus: Infectious Mononucleosis | 118 | ||
Clinical Features | 118 | ||
Pathologic Features | 120 | ||
Differential Diagnosis | 120 | ||
Cytomegaloviral Lymphadenitis | 122 | ||
Clinical Features | 122 | ||
Pathologic Features | 122 | ||
Differential Diagnosis | 122 | ||
Herpes Simplex Viral Lymphadenitis | 124 | ||
2 Lymphomas | 211 | ||
7 Small B-Cell Lymphomas | 213 | ||
■ Introduction | 213 | ||
■ Small Lymphocytic Lymphoma | 213 | ||
Clinical Features | 216 | ||
Pathological Features | 216 | ||
Morphology | 216 | ||
Immunophenotype | 219 | ||
Genetics | 219 | ||
Transformation | 219 | ||
Differential Diagnosis | 220 | ||
Prognosis and Therapy | 221 | ||
■ Mantle Cell Lymphoma | 222 | ||
Clinical Features | 222 | ||
Morphology | 222 | ||
Involvement of Extranodal Sites | 226 | ||
Phenotype | 227 | ||
Genetics | 229 | ||
Differential Diagnosis | 231 | ||
Prognosis and Therapy | 231 | ||
■ Follicular Lymphoma | 231 | ||
Clinical Features | 231 | ||
Pathologic Features | 232 | ||
Morphology | 232 | ||
Phenotype | 237 | ||
Genetics | 240 | ||
Follicular Lymphoma in Unusual Circumstances and in Extranodal Sites | 242 | ||
Pediatric-Type Follicular Lymphoma | 242 | ||
Large B-Cell Lymphoma and Follicular Lymphoma With IRF4 Rearrangement | 243 | ||
Primary Cutaneous Follicle Center Cell Lymphoma (FCL) | 243 | ||
Follicular Lymphoma in the Gastrointestinal Tract and Duodenal-Type Follicular Lymphoma | 245 | ||
Bone Marrow and Spleen Involvement | 245 | ||
In Situ Follicular Neoplasia | 245 | ||
Transformation of Follicular Lymphomas | 245 | ||
Differential Diagnosis | 247 | ||
Prognosis and Therapy | 248 | ||
■ Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue | 248 | ||
Clinical Features | 248 | ||
Pathologic Features | 249 | ||
Morphology | 249 | ||
Immunophenotype | 253 | ||
Genetics | 253 | ||
Prognosis and Therapy | 254 | ||
Differential Diagnosis | 254 | ||
■ Splenic Marginal Zone Lymphoma | 254 | ||
Clinical Features | 255 | ||
Pathologic Features | 255 | ||
Morphology | 255 | ||
Phenotype | 256 | ||
Genetics | 259 | ||
Differential Diagnosis | 259 | ||
Prognosis and Therapy | 260 | ||
■ Nodal Marginal Zone B-Cell Lymphoma | 260 | ||
Clinical Features | 260 | ||
Pathologic Features | 261 | ||
Morphology | 261 | ||
Phenotype | 262 | ||
Genetics | 264 | ||
Differential Diagnosis | 264 | ||
Prognosis and Therapy | 265 | ||
■ Lymphoplasmacytic Lymphoma | 265 | ||
Clinical Features | 265 | ||
Pathologic Features | 266 | ||
Morphology | 266 | ||
Phenotype | 266 | ||
Genetics | 266 | ||
Differential Diagnosis | 268 | ||
Prognosis and Therapy | 270 | ||
Suggested Reading | 270.e1 | ||
General | 270.e1 | ||
Small Lymphocytic Lymphoma | 270.e1 | ||
Mantle Cell Lymphoma | 270.e1 | ||
Follicular Lymphoma | 270.e1 | ||
Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue | 270.e2 | ||
Splenic Marginal Zone Lymphoma | 270.e2 | ||
Nodal Marginal Zone B-Cell Lymphoma | 270.e2 | ||
Lymphoplasmacytic Lymphoma | 270.e3 | ||
8 Diffuse Aggressive B-Cell Lymphomas | 271 | ||
■ Introduction | 271 | ||
■ Diffuse Large B-Cell Lymphoma, Not Otherwise Specified | 271 | ||
Clinical Features | 271 | ||
Pathologic Features | 271 | ||
Histopathology | 271 | ||
Immunophenotype | 274 | ||
Molecular Genetics | 276 | ||
Differential Diagnosis | 276 | ||
Prognosis and Therapy | 278 | ||
■ T-Cell/Histiocyte-Rich Large B-Cell Lymphoma | 278 | ||
Clinical Features | 278 | ||
Pathologic Features | 278 | ||
Histopathology | 278 | ||
Immunophenotype | 278 | ||
Molecular Genetics | 280 | ||
Differential Diagnosis | 280 | ||
Prognosis and Therapy | 280 | ||
■ Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System | 280 | ||
Clinical Features | 280 | ||
Pathologic Features | 280 | ||
Histopathology | 280 | ||
Immunophenotype | 281 | ||
Molecular Genetics | 281 | ||
Differential Diagnosis | 281 | ||
Prognosis and Therapy | 281 | ||
■ Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type | 282 | ||
Clinical Features | 282 | ||
Pathologic Features | 282 | ||
Histopathology | 282 | ||
Immunophenotype | 282 | ||
Molecular Genetics | 282 | ||
Differential Diagnosis | 282 | ||
Prognosis and Therapy | 283 | ||
■ Epstein-Barr Virus–Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified | 283 | ||
Clinical Features | 283 | ||
Pathologic Features | 284 | ||
Histopathology | 284 | ||
Immunophenotype | 284 | ||
Molecular Genetics | 284 | ||
Differential Diagnosis | 284 | ||
Prognosis and Therapy | 284 | ||
■ Epstein-Barr Virus–Positive Mucocutaneous Ulcer | 285 | ||
Clinical Features | 285 | ||
Pathologic Features | 285 | ||
Histopathology | 285 | ||
Immunophenotype | 285 | ||
Molecular Genetics | 286 | ||
Differential Diagnosis | 286 | ||
Prognosis and Therapy | 286 | ||
■ Diffuse Large B-Cell Lymphoma Associated With Chronic Inflammation | 286 | ||
Clinical Features | 286 | ||
Pathologic Features | 287 | ||
Histopathology | 287 | ||
Immunophenotype | 287 | ||
Molecular Genetics | 287 | ||
Differential Diagnosis | 287 | ||
Prognosis and Therapy | 287 | ||
■ Primary Mediastinal Large B-Cell Lymphoma | 288 | ||
Clinical Features | 288 | ||
Pathologic Features | 288 | ||
Histopathology | 288 | ||
Immunophenotype | 288 | ||
Molecular Genetics | 288 | ||
Differential Diagnosis | 289 | ||
Prognosis and Therapy | 290 | ||
■ Intravascular Large B-Cell Lymphoma | 290 | ||
Clinical Features | 290 | ||
Pathologic Features | 291 | ||
Histopathology | 291 | ||
Immunophenotype | 292 | ||
Molecular Genetics | 292 | ||
Differential Diagnosis | 292 | ||
Prognosis and Therapy | 292 | ||
■ ALK-Positive Large B-Cell Lymphoma | 292 | ||
Clinical Features | 292 | ||
Pathologic Features | 292 | ||
Histopathology | 292 | ||
Immunophenotype | 293 | ||
Molecular Genetics | 293 | ||
Differential Diagnosis | 293 | ||
Prognosis and Therapy | 293 | ||
■ Lymphomatoid Granulomatosis | 293 | ||
Clinical Features | 293 | ||
Pathologic Features | 295 | ||
Histopathology | 295 | ||
Immunophenotype | 295 | ||
Molecular Genetics | 295 | ||
Differential Diagnosis | 295 | ||
Prognosis and Therapy | 296 | ||
■ Plasmablastic Lymphoma | 296 | ||
Clinical Features | 296 | ||
Pathologic Features | 297 | ||
Histopathology | 297 | ||
Immunophenotype | 297 | ||
Molecular Genetics | 298 | ||
Differential Diagnosis | 298 | ||
Prognosis and Therapy | 298 | ||
■ Burkitt Lymphoma | 298 | ||
Clinical Features | 298 | ||
Pathologic Features | 299 | ||
Histopathology | 299 | ||
Immunophenotype | 299 | ||
Molecular Genetics | 299 | ||
Differential Diagnosis | 301 | ||
Prognosis and Therapy | 301 | ||
■ Burkitt-Like Lymphoma With 11q Aberration | 302 | ||
Clinical Features | 302 | ||
Pathologic Features | 302 | ||
Histopathology | 302 | ||
Immunophenotype | 302 | ||
Molecular Genetics | 302 | ||
Differential Diagnosis | 302 | ||
Prognosis and Therapy | 302 | ||
■ High-Grade B-Cell Lymphoma | 302 | ||
Clinical Features | 303 | ||
Pathologic Features | 303 | ||
Histopathology | 303 | ||
Immunophenotype | 303 | ||
Molecular Genetics | 304 | ||
Differential Diagnosis | 304 | ||
Prognosis and Therapy | 305 | ||
Suggested Reading | 305.e1 | ||
Diffuse Large B-Cell Lymphoma | 305.e1 | ||
T Cell/Histiocyte-Rich Large B-Cell Lymphoma | 305.e1 | ||
Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System | 305.e1 | ||
Primary Cutaneous DLBCL, Leg Type | 305.e2 | ||
EBV-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified | 305.e2 | ||
EBV-Positive Mucocutaneous Ulcer | 305.e2 | ||
DLBCL Associated With Chronic Inflammation | 305.e2 | ||
Primary Mediastinal Large B-Cell Lymphoma | 305.e2 | ||
Intravascular Large B-Cell Lymphoma | 305.e3 | ||
ALK-Positive Large B-Cell Lymphoma | 305.e3 | ||
Lymphomatoid Granulomatosis | 305.e3 | ||
Plasmablastic Lymphoma | 305.e3 | ||
Burkitt Lymphoma | 305.e4 | ||
Burkitt-Like Lymphoma With 11q Aberration | 305.e4 | ||
High-Grade B-Cell Lymphoma | 305.e4 | ||
9 Peripheral T-Cell Lymphomas | 306 | ||
■ Introduction | 306 | ||
■ Angioimmunoblastic T-Cell Lymphoma and Other Nodal Lymphomas of T Follicular Helper Cell Origin | 306 | ||
Clinical Features | 306 | ||
Pathologic Features | 306 | ||
Microscopic Findings | 306 | ||
Immunophenotype | 307 | ||
Molecular and Cytogenetic Findings | 307 | ||
Differential Diagnosis | 310 | ||
■ Other Nodal Lymphomas of T Follicular Helper Cell Origin | 310 | ||
■ Anaplastic Large-Cell Lymphoma—ALK Positive and ALK Negative | 310 | ||
Clinical Features | 311 | ||
Pathologic Features | 311 | ||
Microscopic Findings | 311 | ||
Immunophenotype | 313 | ||
Molecular and Cytogenetic Findings | 313 | ||
Differential Diagnosis | 313 | ||
■ Peripheral T-Cell Lymphoma, Not Otherwise Specified | 315 | ||
Clinical Features | 315 | ||
Pathologic Features | 315 | ||
Microscopic Findings | 315 | ||
Immunophenotype | 315 | ||
Molecular and Cytogenetic Findings | 315 | ||
Differential Diagnosis | 317 | ||
■ Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type | 317 | ||
Clinical Features | 317 | ||
Pathologic Features | 317 | ||
Microscopic Findings | 317 | ||
Immunophenotype | 318 | ||
Molecular and Cytogenetic Findings | 318 | ||
Differential Diagnosis | 320 | ||
■ Subcutaneous Panniculitis–Like T-Cell Lymphoma | 320 | ||
Clinical Features | 320 | ||
Pathologic Features | 321 | ||
Microscopic Findings | 321 | ||
Immunophenotype | 321 | ||
Molecular and Cytogenetic Findings | 321 | ||
Differential Diagnosis | 321 | ||
■ Hepatosplenic T-Cell Lymphoma | 323 | ||
Clinical Features | 323 | ||
Pathologic Features | 323 | ||
Microscopic Findings | 323 | ||
Immunophenotype | 323 | ||
Molecular and Cytogenetic Findings | 323 | ||
Differential Diagnosis | 325 | ||
■ Intestinal T-Cell Lymphoma | 325 | ||
Clinical Features | 325 | ||
Pathologic Features | 326 | ||
Microscopic Findings | 326 | ||
Immunophenotype | 326 | ||
Molecular and Cytogenetic Findings | 326 | ||
Differential Diagnosis | 328 | ||
■ Mycosis Fungoides and Sézary Syndrome | 328 | ||
Clinical Features | 328 | ||
Pathologic Features | 328 | ||
Microscopic Findings | 328 | ||
Immunophenotype | 331 | ||
Molecular and Cytogenetic Findings | 331 | ||
Differential Diagnosis | 331 | ||
■ Primary Cutaneous CD30+ T-Cell Lymphoproliferative Disorders | 331 | ||
Clinical Features | 331 | ||
Pathologic Features | 332 | ||
Microscopic Findings | 332 | ||
Immunophenotype | 334 | ||
Molecular and Cytogenetic Findings | 334 | ||
Differential Diagnosis | 334 | ||
■ Primary Cutaneous Peripheral T-Cell Lymphomas: Rare Subtypes | 334 | ||
Clinical Features | 334 | ||
Pathologic Features | 334 | ||
Microscopic Findings | 334 | ||
Immunophenotype | 336 | ||
Molecular and Cytogenetic Findings | 337 | ||
Differential Diagnosis | 338 | ||
■ Epstein-Barr Virus–Positive T-Cell Lymphoproliferative Disorders of Childhood | 338 | ||
Clinical Features | 338 | ||
Pathologic Features | 339 | ||
Microscopic Findings | 339 | ||
Immunophenotype | 339 | ||
Molecular and Cytogenetic Findings | 339 | ||
Differential Diagnosis | 339 | ||
Suggested Readings | 339.e1 | ||
Angioimmunoblastic T-Cell Lymphoma | 339.e1 | ||
Anaplastic Large Cell Lymphoma | 339.e1 | ||
Peripheral T-Cell Lymphoma, NOS | 339.e1 | ||
Extranodal NK/T-Cell Lymphoma, Nasal Type | 339.e1 | ||
Subcutaneous Panniculitis–like T-Cell Lymphoma | 339.e2 | ||
Hepatosplenic T-Cell Lymphoma | 339.e2 | ||
Intestinal T-Cell Lymphoma | 339.e2 | ||
Mycosis Fungoides and Sézary Syndrome | 339.e2 | ||
Primary Cutaneous CD30+ T-Cell Lymphoproliferative Disorders | 339.e2 | ||
Primary Cutaneous Peripheral T-Cell Lymphomas, Rare Subtypes | 339.e3 | ||
Epstein-Barr Virus–Positive T-Cell Lymphoproliferative Disorders of Childhood | 339.e3 | ||
10 Immunodeficiency-Related Lymphoproliferative Disorders | 340 | ||
■ Introduction | 340 | ||
■ Posttransplantation Lymphoproliferative Disorders | 340 | ||
Clinical Features | 340 | ||
Pathologic Features | 340 | ||
Prognosis and Therapy | 344 | ||
Immunophenotype | 344 | ||
Genetic Features | 344 | ||
■ Human Immunodeficiency Virus–Related Lymphoproliferative Disorders | 344 | ||
Introduction and Definition | 344 | ||
Clinical Features | 346 | ||
Pathologic Features | 346 | ||
■ Diffuse Large B-Cell Lymphomas | 347 | ||
■ Burkitt Lymphoma | 347 | ||
■ Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma) | 350 | ||
■ Peripheral T/NK-Cell Lymphomas | 350 | ||
■ Mycosis Fungoides | 350 | ||
■ Primary Effusion Lymphoma and Other Kaposi Sarcoma–Associated, Herpesvirus-Related Non-Hodgkin Lymphomas | 351 | ||
■ Plasmablastic Lymphoma | 355 | ||
■ Polymorphic B-Cell Lymphoma (PTLD-Like) | 355 | ||
■ Multiple Myeloma | 357 | ||
■ Classic Hodgkin Lymphoma | 357 | ||
■ Bone Marrow Involvement in AIDS-Related Lymphoma | 357 | ||
Prognosis and Therapy | 358 | ||
■ Lymphoproliferative Diseases Associated With Primary Immune Disorders | 358 | ||
■ Ataxia Telangiectasia | 358 | ||
■ Autoimmune Lymphoproliferative Syndrome (ALPS) | 358 | ||
■ Lymphoproliferative Disorders in Patients Treated With Immunosuppressive Drugs for Autoimmune Diseases or Related Conditions (Immunomodulator Agent–Related Lymphoproliferative Disorders) | 360 | ||
■ Common Variable Immunodeficiency | 361 | ||
Suggested Reading | 362.e1 | ||
General | 362.e1 | ||
Posttransplant Lymphoproliferative Disorders (PTLD) | 362.e1 | ||
AIDS-Related Lymphomas | 362.e1 | ||
Primary and Acquired Immune Disorders | 362.e1 | ||
Mucocutaneous Ulcer | 362.e1 | ||
11 Hodgkin Lymphoma* | 363 | ||
■ Introduction | 363 | ||
■ Definition | 363 | ||
■ Etiology and Postulated Cell of Origin | 363 | ||
Clinical Features | 364 | ||
Classical Hodgkin Lymphoma | 364 | ||
Clinical Presentation | 364 | ||
CHL in Immunosuppressed Patients | 364 | ||
Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 364 | ||
Staging of Hodgkin Lymphoma | 364 | ||
Prognosis and Therapy | 365 | ||
Treatment | 366 | ||
Pathologic Features | 366 | ||
Histopathologic Classification of Hodgkin Lymphoma | 366 | ||
Classical Hodgkin Lymphoma | 367 | ||
Reed-Sternberg Cells and Variants | 367 | ||
■ Nodular Sclerosis Classical Hodgkin Lymphoma | 369 | ||
Clinical Features | 369 | ||
Microscopic Features | 369 | ||
■ Mixed Cellularity Classical Hodgkin Lymphoma | 371 | ||
Clinical Features | 373 | ||
Microscopic Features | 373 | ||
■ Lymphocyte-Rich Classical Hodgkin Lymphoma | 375 | ||
Clinical Features | 375 | ||
Microscopic Features | 375 | ||
■ Lymphocyte-Depleted Classical Hodgkin Lymphoma | 375 | ||
Clinical Features | 376 | ||
Histologic Features | 377 | ||
Diagnosis of Relapsed CHL and Diagnosis at Extranodal Sites | 378 | ||
Ancillary Studies | 379 | ||
Immunophenotype | 379 | ||
Immunophenotype of the Reed-Sternberg Cell in Classical Hodgkin Lymphoma | 379 | ||
Immunophenotype of Background Lymphocytes | 380 | ||
Genetic Features of Classical Hodgkin Lymphoma | 381 | ||
Differential Diagnosis | 381 | ||
Primary Mediastinal Large B-Cell Lymphoma (PMLBCL) and B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Dlbcl and Classical Hodgkin Lymphoma (BCL-U) | 382 | ||
Diffuse Large B-Cell Lymphoma | 383 | ||
EBV-Associated Lymphoproliferations in Immunosuppressed Hosts | 383 | ||
Anaplastic Large-Cell Lymphoma | 383 | ||
Other Types of Peripheral T-Cell Lymphomas | 384 | ||
■ Other Non-Hodgkin Lymphomas With H/RS-Like Cells and Composite Lymphomas | 384 | ||
Non-Lymphoid Neoplasms: Metastatic Carcinomas, Melanoma, Primary Mediastinal Seminoma | 385 | ||
Reactive Disorders | 385 | ||
Granulomatous Lymphadenitis | 385 | ||
Other Reactive Disorders | 385 | ||
■ Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 386 | ||
Clinical Findings | 386 | ||
Prognosis and Therapy | 386 | ||
Pathologic Features | 386 | ||
Lymphocyte-Predominant (LP) Cells | 386 | ||
Microscopic Features | 387 | ||
Ancillary Studies | 388 | ||
Immunophenotype of LP Cells | 388 | ||
Genetic Features of Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 389 | ||
Transformation of NLPHL to Large-Cell Lymphoma | 390 | ||
Differential Diagnosis | 390 | ||
Nodular Lymphocyte-Predominant Hodgkin Lymphoma Versus Progressive Transformation of Germinal Centers | 391 | ||
Lymphocyte-Rich Classical Hodgkin Lymphoma Versus Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 391 | ||
T-Cell/Histiocyte–Rich Large B-Cell Lymphoma Versus Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 392 | ||
Follicular Lymphoma Versus Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 392 | ||
Mantle Cell Lymphoma Versus Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 392 | ||
Suggested Readings | 393.e1 | ||
Hodgkin Lymphoma | 393.e1 | ||
Hodgkin Lymphoma in Immunodeficient States | 393.e1 | ||
Classical Hodgkin Lymphoma | 393.e1 | ||
Nodular Sclerosis Classical Hodgkin Lymphoma | 393.e1 | ||
Lymphocyte-Rich Classical Hodgkin Lymphoma | 393.e1 | ||
Lymphocyte-Depleted Classical Hodgkin Lymphoma | 393.e1 | ||
Differential Diagnosis of Classical Hodgkin Lymphoma | 393.e1 | ||
Nodular Lymphocyte-Predominant Hodgkin Lymphoma | 393.e2 | ||
3 Leukemias, Chronic Myeloproliferative Neoplasms, and Myelodysplasia | 395 | ||
12 B-Cell Leukemias of Mature Lymphocytes | 397 | ||
■ Introduction | 397 | ||
■ Chronic Lymphocytic Leukemia | 397 | ||
Clinical Features | 397 | ||
Pathologic Features | 397 | ||
Morphology | 397 | ||
Immunophenotype | 399 | ||
Molecular Genetics | 401 | ||
Differential Diagnosis | 404 | ||
Prognosis and Therapy | 405 | ||
■ Monoclonal B-cell lymphocytosis (MBL) | 406 | ||
■ B-Cell Prolymphocytic Leukemia | 406 | ||
Clinical Features | 406 | ||
Pathologic Features | 407 | ||
Morphology | 407 | ||
Immunophenotype | 408 | ||
Molecular Genetics | 408 | ||
Differential Diagnosis | 408 | ||
Prognosis and Therapy | 408 | ||
■ Hairy Cell Leukemia | 408 | ||
Clinical Features | 408 | ||
Pathologic Features | 408 | ||
Morphology | 408 | ||
Immunophenotype | 410 | ||
Molecular Genetics | 410 | ||
Differential Diagnosis | 410 | ||
Prognosis and Therapy | 411 | ||
■ Hairy Cell Leukemia Variant | 411 | ||
Clinical Features | 411 | ||
Pathologic Features | 411 | ||
Morphology | 411 | ||
Immunophenotype | 413 | ||
Molecular Genetics | 413 | ||
Differential Diagnosis | 413 | ||
Prognosis and Therapy | 413 | ||
Suggested Reading | 413.e1 | ||
Chronic Lymphocytic Leukemia | 413.e1 | ||
Monoclonal B-Cell Lymphocytosis (MBL) | 413.e1 | ||
Prolymphocytic Leukemia | 413.e2 | ||
Hairy Cell Leukemia | 413.e2 | ||
Hairy Cell Leukemia Variant | 413.e2 | ||
13 Mature T-Cell and Natural Killer–Cell Leukemias | 414 | ||
■ Introduction | 414 | ||
■ Evaluation of Peripheral Blood in Mature T-Cell Leukemias | 414 | ||
Morphologic Evaluation | 414 | ||
Flow Cytometric Immunophenotyping | 414 | ||
Antigen Receptor Gene Rearrangement Studies | 415 | ||
Cytogenetic Analysis | 415 | ||
■ Specific Types of Mature T-Cell and Natural Killer–Cell Leukemias | 415 | ||
Large Granular Lymphocytic Leukemia | 415 | ||
Clinical Features | 415 | ||
Morphologic Findings | 417 | ||
Immunophenotype | 417 | ||
Genetic and Cytogenetic Findings | 417 | ||
Differential Diagnosis | 418 | ||
Reactive Lymphocytosis | 418 | ||
Posttransplantation Large Granular Lymphocyte Proliferations | 418 | ||
Sézary Syndrome and Mycosis Fungoides | 418 | ||
Clinical Features | 418 | ||
Morphologic Findings | 419 | ||
Immunophenotypic Findings | 420 | ||
Cytogenetic Findings | 420 | ||
Molecular Genetic Findings | 420 | ||
Differential Diagnosis | 421 | ||
Adult T-Cell Leukemia–Lymphoma Associated With Human T-Lymphotropic Virus 1 | 421 | ||
Clinical Features | 421 | ||
Morphologic Findings | 422 | ||
Immunophenotypic Findings | 422 | ||
Cytogenetic Findings | 422 | ||
Molecular Genetic Findings | 423 | ||
Differential Diagnosis | 423 | ||
T-Cell Prolymphocytic Leukemia | 423 | ||
Clinical Features | 423 | ||
Morphologic Findings | 424 | ||
Immunophenotypic Findings | 424 | ||
Cytogenetic Findings | 424 | ||
Molecular Genetic Findings | 425 | ||
Differential Diagnosis | 425 | ||
Aggressive Natural Killer–Cell Leukemia | 425 | ||
Clinical Features | 425 | ||
Morphologic Findings | 425 | ||
Immunophenotypic Findings | 426 | ||
Cytogenetic Findings | 426 | ||
Differential Diagnosis | 426 | ||
Leukemic Phase of Other Peripheral T-Cell Lymphomas | 427 | ||
Lymphocytic Variant Hypereosinophilic Syndromes | 427 | ||
Clinical Features | 427 | ||
Morphologic Findings | 428 | ||
Immunophenotypic Findings | 428 | ||
Genetic and Cytogenetic Findings | 428 | ||
Differential Diagnosis | 428 | ||
Suggested Reading | 428.e1 | ||
General | 428.e1 | ||
Large Granular Lymphocytic Leukemia and Related Disorders | 428.e1 | ||
Sézary Syndrome and Mycosis Fungoides | 428.e1 | ||
Adult T-Cell Leukemia/Lymphoma and HTLV-1 | 428.e1 | ||
T-Cell Prolymphocytic Leukemia | 428.e1 | ||
Aggressive NK Cell Leukemia | 428.e1 | ||
Lymphocytic Variant Hypereosinophilic Syndromes | 428.e1 | ||
14 Acute Myeloid Leukemia | 429 | ||
■ Introduction and Classification | 429 | ||
■ De Novo Acute Myeloid Leukemias With Recurrent Genetic Abnormalities | 429 | ||
Acute Myeloid Leukemia With t(8;21)(q22;q22.1) RUNX1-RUNX1T1 | 429 | ||
Acute Myeloid Leukemia With inv(16)(p13.1;q22) or t(16;16)(p13.1;q22); CBFB-MYH11 | 432 | ||
Acute Promyelocytic Leukemia With PML-RARA | 434 | ||
Acute Myeloid Leukemia With t(9;11) (p21.3;q23.3) KMT2A-MLLT3 | 436 | ||
Acute Myeloid Leukemia With t(6;9) (p23;q34.1); DEK-NUP214 | 438 | ||
Acute Myeloid Leukemia With inv(3)(q21.3;q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM (EVI1) | 439 | ||
Acute Myeloid Leukemia (Megakaryoblastic) With t(1;22)(p13.3;q13.1); RBM15-MKL1 | 441 | ||
Acute Myeloid Leukemia With BCR-ABL1 | 441 | ||
Acute Myeloid Leukemia With Gene Mutations | 443 | ||
Acute Myeloid Leukemia With Mutated NPM1 | 444 | ||
Acute Myeloid Leukemia With Biallelic Mutations of CEBPA | 444 | ||
Acute Myeloid Leukemia With Mutated RUNX1 | 445 | ||
■ Acute Myeloid Leukemia With Myelodysplasia-Related Changes | 445 | ||
■ Therapy-Related Myeloid Neoplasms | 448 | ||
■ Acute Myeloid Leukemia, Not Otherwise Specified, and the French-American-British Cooperative Group Classification of Acute Myeloid Leukemia | 450 | ||
■ Myeloid Sarcoma | 456 | ||
■ Myeloid Proliferations of Down Syndrome | 457 | ||
■ Myeloid Neoplasms With Germline Predisposition | 458 | ||
■ Blastic Plasmacytoid Dendritic Cell Tumor (BPDC) | 459 | ||
■ Growth Factor Changes That CAN Mimic Acute Myeloid Leukemia | 461 | ||
■ Post-Therapy Changes in Acute Myeloid Leukemia | 462 | ||
Changes Associated With Myeloablative Therapy | 462 | ||
Marrow After Recovery From Acute Myeloid Leukemia Therapy | 462 | ||
■ Acknowledgment | 466 | ||
Suggested Reading | 466.e1 | ||
Acute Myeloid Leukemia Classification | 466.e1 | ||
Acute Myeloid Leukemia With t(8;21)(q22;q22.1) RUNX1-RUNX1T1 | 466.e1 | ||
Acute Myeloid Leukemia With inv(16) (p13.1;q22) or t(16;16) (p13.1;q22); CBFB-MYH11 | 466.e1 | ||
Acute Promyelocytic Leukemia With PML-RARA | 466.e1 | ||
Acute Myeloid Leukemia With t(9;11)(p21.3;q23.3) KMT2A-MLLT3 | 466.e1 | ||
Acute Myeloid Leukemia With t(6;9) (p23;q34) DEK-NUP214 | 466.e2 | ||
Acute Myeloid Leukemia With inv(3)(q21.3;q26.2) or t(3;3)(q21.3;q26.2); RPN1-EVI1 | 466.e2 | ||
Acute Myeloid Leukemia (Megakaryoblastic) With t(1;22)(p13.3;q13.1); RBM15-MKL1 | 466.e2 | ||
Acute Myeloid Leukemia With BCR-ABL1 | 466.e2 | ||
Acute Myeloid Leukemia With Gene Mutations | 466.e2 | ||
Acute Myeloid Leukemia With Mutated NPM1 | 466.e3 | ||
Acute Myeloid Leukemia With Biallelic Mutations of CEBPA | 466.e3 | ||
Acute Myeloid Leukemia With Mutated RUNX1 | 466.e3 | ||
Acute Myeloid Leukemia With Myelodysplasia-Related Changes | 466.e3 | ||
Therapy-Related Acute Myeloid Leukemia | 466.e3 | ||
Acute Myeloid Leukemia, Not Otherwise Categorized, and the French-American-British Cooperative Group Classification of Acute Myeloid Leukemia | 466.e4 | ||
Myeloid Neoplasms With Germline Predisposition | 466.e4 | ||
Myeloid Proliferations of Down Syndrome | 466.e4 | ||
Blastic Plasmacytoid Dendritic Cell Tumor | 466.e4 | ||
Growth Factor Changes That Can Mimic Acute Myeloid Leukemia | 466.e4 | ||
Post-Therapy Changes in Acute Myeloid Leukemia | 466.e5 | ||
Marrow After Recovery from Acute Myeloid Leukemia Therapy | 466.e5 | ||
15 Precursor Lymphoid Neoplasms | 467 | ||
■ Introduction | 467 | ||
Clinical Features | 467 | ||
Pathologic Features | 467 | ||
Gross Findings | 467 | ||
Microscopic Findings | 467 | ||
Ancillary Studies | 468 | ||
Immunophenotypic Analysis | 468 | ||
Molecular and Cytogenetic Features | 473 | ||
Differential Diagnosis | 479 | ||
Prognosis and Therapy | 479 | ||
Suggested Reading | 480.e1 | ||
16 Acute Undifferentiated Leukemia and Mixed-Phenotype Acute Leukemias | 481 | ||
■ Introduction | 481 | ||
■ Acute Undifferentiated Leukemias | 481 | ||
Clinical Features | 481 | ||
Pathologic Features | 481 | ||
Microscopic Features | 481 | ||
Ancillary Studies | 481 | ||
Immunophenotypic Findings | 481 | ||
Molecular and Cytogenetic Findings | 482 | ||
Differential Diagnosis | 482 | ||
Prognosis and Therapy | 482 | ||
■ Mixed-Phenotype Acute Leukemias | 482 | ||
Clinical Features | 482 | ||
Pathologic Features | 483 | ||
Microscopic Findings | 483 | ||
Ancillary Studies | 483 | ||
Immunophenotypic Findings | 483 | ||
Molecular and Cytogenetic Findings | 484 | ||
Differential Diagnosis | 486 | ||
Prognosis and Therapy | 487 | ||
Suggested Reading | 487.e1 | ||
Acute Undifferentiated Leukemia | 487.e1 | ||
Mixed Phenotype Acute Leukemia | 487.e1 | ||
17 Myeloproliferative and “Overlap” Myelodysplastic/Myeloproliferative Neoplasms | 488 | ||
■ Introduction | 488 | ||
■ Myeloproliferative Neoplasms | 489 | ||
Chronic Myeloid Leukemia (CML) | 489 | ||
Clinical Features | 490 | ||
Pathologic Features | 490 | ||
Chronic Phase | 490 | ||
Peripheral Blood | 490 | ||
Bone Marrow | 490 | ||
Ancillary Studies | 492 | ||
Accelerated Phase | 494 | ||
Blast Phase | 494 | ||
Myeloid Blast Phase | 496 | ||
Lymphoid Blast Phase | 496 | ||
Bilineal or Biphenotypic Blast Phase | 496 | ||
Extramedullary Blast Phase | 496 | ||
Differential Diagnosis | 497 | ||
Differential Diagnosis of Chronic Phase | 499 | ||
Essential Thrombocythemia | 500 | ||
Differential Diagnosis of Accelerated Phase and Blast Phase | 500 | ||
Prognosis and Therapy (Box 17.1) | 501 | ||
BCR-ABL–Negative Myeloproliferative Neoplasms | 503 | ||
Polycythemia Vera | 503 | ||
Clinical Features | 503 | ||
Pathologic Features | 504 | ||
Blood and Bone Marrow: Polycythemic Phase | 504 | ||
Molecular | 506 | ||
Cytogenetics | 506 | ||
Blood and Marrow, Spent Phase, Post-Polycythemic Myelofibrosis | 506 | ||
Differential Diagnosis | 506 | ||
Prognosis and Therapy | 506 | ||
Essential Thrombocythemia | 506 | ||
Clinical Features | 506 | ||
Pathologic Features | 507 | ||
Blood and Bone Marrow | 507 | ||
Molecular and Ancillary Studies | 508 | ||
Differential Diagnosis | 509 | ||
Prognosis and Therapy | 510 | ||
Primary Myelofibrosis | 510 | ||
Clinical Features | 510 | ||
Pathologic Features | 511 | ||
Diagnosis, General | 511 | ||
Differential Diagnosis: Prefibrotic Phase | 511 | ||
4 Disorders of Histiocytes, Mast Cells, Plasma Cells, Spleen, and Ancillary Techniques | 565 | ||
19 Disorders of Histiocytes*The authors gratefully acknowledge the contribution of Dr. Ronald Jaffe, who authored this chapter in previous editions and critically reviewed the current edition. | 567 | ||
■ Derivation of Histiocytes | 567 | ||
■ Identification of Histiocytes | 570 | ||
■ Physiologic Excess of Histiocytes | 571 | ||
Macrophages | 571 | ||
Dendritic Cells | 572 | ||
■ Pathologic Excess of Macrophages | 572 | ||
Infection and Foreign Materials | 572 | ||
Xanthomas and Xanthomatous Inflammation | 572 | ||
■ Functional Defects of Histiocytes | 576 | ||
■ Hemophagocytic Lymphohistiocytosis | 577 | ||
■ Secondary Hemophagocytic Lymphohistiocytosis Including Macrophage Activation Syndrome | 577 | ||
■ Genetic Forms of Hemophagocytic Lymphohistiocytosis, Including Familial HLH | 578 | ||
Clinical Features | 578 | ||
Pathologic Features | 580 | ||
Gross Findings | 580 | ||
Microscopic Findings | 580 | ||
Ancillary Studies | 581 | ||
Molecular and Cytogenetic Features | 581 | ||
Immunohistochemistry | 583 | ||
Fine-Needle Aspiration Biopsy | 583 | ||
Differential Diagnosis | 583 | ||
Prognosis and Therapy | 583 | ||
■ Cytophagic Histiocytic Panniculitis | 583 | ||
Classification of the Histiocytic Proliferations | 584 | ||
■ Langerhans Cell Histiocytosis | 584 | ||
Clinical Features | 584 | ||
Radiologic Features | 586 | ||
Bone and Adjacent Soft Tissue | 586 | ||
Pathologic Features | 587 | ||
Gross Findings | 587 | ||
Microscopic Findings | 588 | ||
Ancillary Studies | 590 | ||
Immunohistochemistry | 590 | ||
Molecular and Cytogenic Features | 590 | ||
Ultrastructure Features | 591 | ||
Fine-Needle Aspiration | 591 | ||
Differential Diagnosis | 591 | ||
Prognosis and Therapy | 594 | ||
■ Non–Langerhans Cell Histiocytosis | 595 | ||
Erdheim-Chester Disease, Including Adult Xanthogranuloma Family Lesions | 595 | ||
Clinical Features | 595 | ||
Radiologic Features | 595 | ||
Pathologic Features | 595 | ||
Gross Findings | 595 | ||
Microscopy Findings | 595 | ||
Erdheim-Chester Disease Pathologic Features | 596 | ||
Ancillary Studies | 596 | ||
Immunohistochemistry | 596 | ||
Molecular and Cytogenetic Features | 598 | ||
Differential Diagnosis | 598 | ||
Prognosis and Therapy | 598 | ||
■ Juvenile Xanthogranuloma Family | 598 | ||
Clinical Features | 598 | ||
Radiologic Features | 598 | ||
Pathologic Features | 599 | ||
Gross Findings | 599 | ||
Microscopic Findings | 599 | ||
Ancillary Studies | 600 | ||
Immunohistochemistry | 600 | ||
Molecular and Cytogenetic Features | 601 | ||
Ultrastructural Features | 601 | ||
Fine-Needle Aspiration Biopsy | 601 | ||
Differential Diagnosis | 601 | ||
Prognosis and Therapy | 601 | ||
■ Reticulohistiocytoma and Multicentric Reticulohistiocytosis | 602 | ||
Pathologic Findings | 602 | ||
Prognosis and Therapy | 603 | ||
■ Rosai-Dorfman Disease | 603 | ||
Clinical Features | 603 | ||
Radiologic Features | 603 | ||
Pathologic Features | 603 | ||
Gross Findings | 603 | ||
Microscopic Findings | 603 | ||
Ancillary Studies | 604 | ||
Electron Microscopy | 604 | ||
Molecular and Cytogenic Features | 604 | ||
Immunohistochemistry | 604 | ||
Fine-Needle Aspiration Biopsy | 604 | ||
Differential Diagnosis | 604 | ||
Prognosis and Therapy | 605 | ||
■ Other Non–Langerhans Cell Histiocytoses | 606 | ||
■ Combined Langerhans and Non-Langerhans Histiocytosis | 606 | ||
Langerhans Cell Histiocytosis and Juvenile Xanthogranuloma | 606 | ||
Langerhans Cell Histiocytosis and Erdheim-Chester Disease | 606 | ||
Langerhans Cell Histiocytosis and Rosai-Dorfman Disease | 606 | ||
■ Histiocytosis Following Leukemia, or Follicular Lymphomas | 606 | ||
■ Histiocytic Malignancies | 607 | ||
Histiocytic Sarcoma | 607 | ||
Clinical Features | 607 | ||
Pathologic Features | 607 | ||
Microscopic Findings | 607 | ||
Ancillary Studies | 608 | ||
Molecular and Cytogenetic Features | 608 | ||
Electron Microscopy | 608 | ||
Immunohistochemistry | 609 | ||
Differential Diagnosis | 609 | ||
Prognosis and Therapy | 610 | ||
Histiocytic Sarcoma With Juvenile Xanthogranuloma Phenotype | 610 | ||
Langerhans Cell Sarcoma | 610 | ||
Pathologic Features | 610 | ||
Ancillary Studies | 610 | ||
Molecular and Cytogenetic Features | 610 | ||
Electron Microscopy | 610 | ||
Immunohistochemistry | 610 | ||
Differential Diagnosis | 610 | ||
■ Index | 761 | ||
A | 761 | ||
B | 763 | ||
C | 764 | ||
D | 765 | ||
E | 766 | ||
F | 766 | ||
G | 767 | ||
H | 767 | ||
I | 769 | ||
J | 770 | ||
K | 770 | ||
L | 770 | ||
M | 771 | ||
N | 774 | ||
O | 775 | ||
P | 775 | ||
Q | 777 | ||
R | 777 | ||
S | 778 | ||
T | 779 | ||
U | 780 | ||
V | 780 | ||
W | 780 | ||
X | 780 | ||
Z | 780 | ||
Inside Back Cover | ibc1 |