BOOK
Hematopathology E-Book
Elaine Sarkin Jaffe | Nancy Lee Harris | James Vardiman | Daniel A. Arber | Elias Campo
(2010)
Additional Information
Book Details
Abstract
Introducing HEMATOPATHOLOGY, a definitive new diagnostic reference on diseases of the hematopoietic system by Dr. Elaine S. Jaffe and her fellow editors, all collaborators on the World Health Organization's classification of lymphoid and myeloid disorders. These experts provide you with today's most effective guidance in evaluating specimens from the lymph nodes, bone marrow, peripheral blood, and more, equipping you to deliver more accurate and actionable pathology reports. More than 1,100 high-quality color images mirror the findings you encounter in practice.
- Overcome the toughest diagnostic challenges with authoritative guidance from the world's leading experts.
- Make optimal use of the newest diagnostic techniques, including molecular, immunohistochemical, and genetic studies.
- Compare specimens to more than 1,100 high-quality color images to confirm or challenge your diagnostic interpretations.
Search the full contents online and download any of the images at expertconsult.com.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front cover | cover | ||
| Hematopathology | i | ||
| Copyright page | ii | ||
| Contributors | iii | ||
| Preface | ix | ||
| Table of Contents | xi | ||
| I Technical Aspects | 1 | ||
| 1 Processing of the Lymph Node Biopsy Specimen | 3 | ||
| Chapter Outline | 3 | ||
| Instructions for the Surgeon | 3 | ||
| Gross Processing of the Lymph Node Biopsy by the Pathologist | 4 | ||
| Gross Examination | 4 | ||
| Frozen Sections | 4 | ||
| Cytologic Preparations | 4 | ||
| Sectioning | 6 | ||
| Fixation | 6 | ||
| Contribution of the Histotechnologist | 8 | ||
| Routine Histologic, Histochemical, and Special Stains | 10 | ||
| Choice of Ancillary Studies | 11 | ||
| Reporting the Lymph Node Biopsy | 12 | ||
| References | 13.e1 | ||
| 2 Fine-Needle Aspiration of Lymph Nodes | 14 | ||
| Chapter Outline | 14 | ||
| Fine-Needle Aspiration Specimen Collection and Processing | 15 | ||
| Ancillary Studies | 15 | ||
| Immunocytochemistry | 15 | ||
| Flow Cytometry | 15 | ||
| Molecular Studies | 16 | ||
| Nonneoplastic Aspirates | 17 | ||
| Aspirates of Lymphoid Neoplasms | 18 | ||
| Mature B-Cell Neoplasms | 18 | ||
| Diffuse Large B-Cell Lymphoma, Not Otherwise Specified | 18 | ||
| Cytomorphology. | 18 | ||
| Differential Diagnosis. | 19 | ||
| Follicular Lymphoma | 19 | ||
| Cytomorphology. | 19 | ||
| Grading. | 19 | ||
| II Normal and Reactive Conditions of Hematopoietic Tissues | 95 | ||
| 8 Normal Lymphoid Organs and Tissues | 97 | ||
| CHAPTER OUTLINE | 97 | ||
| Normal Lymphoid Tissues | 97 | ||
| Primary (Central) Lymphoid Tissues | 98 | ||
| Bone Marrow | 98 | ||
| Thymus | 98 | ||
| Secondary (Peripheral) Lymphoid Tissues | 100 | ||
| Lymph Nodes | 100 | ||
| Cortical Area. | 100 | ||
| Paracortex. | 104 | ||
| Lymph Node Vasculature and Conduit System. | 105 | ||
| Spleen | 106 | ||
| White Pulp. | 106 | ||
| Red Pulp. | 106 | ||
| Mucosa-Associated Lymphoid Tissue | 106 | ||
| B-Cell and T-Cell Differentiation | 108 | ||
| Differentiation of Cells of the Adaptive Immune Response | 109 | ||
| B-Cell Differentiation | 109 | ||
| Antigen-Independent B-Cell Differentiation | 109 | ||
| Precursor B Cells. | 109 | ||
| Naïve B Cells. | 110 | ||
| Antigen-Dependent B-Cell Differentiation | 111 | ||
| T-Cell–Independent B-Cell Reaction. | 111 | ||
| T-Cell–Dependent Germinal Center Reaction. | 111 | ||
| Proliferation. | 111 | ||
| Somatic Hypermutation. | 111 | ||
| Selection. | 113 | ||
| Differentiation. | 113 | ||
| Memory B Cells. | 113 | ||
| Plasma Cells. | 114 | ||
| Mucosa-Associated Lymphoid Tissue. | 114 | ||
| T-Cell Differentiation | 114 | ||
| Antigen-Independent T-Cell Differentiation | 114 | ||
| Cortical Thymocytes. | 114 | ||
| Naïve T Cells. | 115 | ||
| Antigen-Dependent T-Cell Differentiation | 115 | ||
| T Immunoblasts. | 115 | ||
| Effector T Cells. | 115 | ||
| Differentiation of Cells of the Innate Immune Response | 115 | ||
| Gamma-Delta T Cells | 117 | ||
| Natural Killer Cells | 117 | ||
| References | 117.e1 | ||
| 9 The Reactive Lymphadenopathies | 118 | ||
| Chapter Outline | 118 | ||
| Follicular and Nodular Patterns | 118 | ||
| Follicular Hyperplasia | 118 | ||
| Differential Diagnosis | 119 | ||
| Monocytoid B-Cell Proliferation | 119 | ||
| Autoimmune Disorders: Rheumatoid Arthritis | 119 | ||
| Luetic Lymphadenitis | 121 | ||
| Hyaline Vascular Castleman’s Disease | 121 | ||
| Histology | 122 | ||
| Immunophenotype | 123 | ||
| Differential Diagnosis | 123 | ||
| Progressive Transformation of Germinal Centers | 125 | ||
| Mantle Zone Hyperplasia | 125 | ||
| Predominantly Sinus Pattern | 126 | ||
| Sinus Histiocytosis | 126 | ||
| Histiocytic Expansion with a Specific Cause | 126 | ||
| Lymphangiograms, Prostheses, and Storage Diseases | 126 | ||
| Whipple’s Disease | 127 | ||
| Vascular Transformation of Sinuses | 127 | ||
| Hemophagocytic Syndrome | 127 | ||
| Interfollicular or Mixed Patterns | 129 | ||
| Paracortical Hyperplasia and Dermatopathic Reaction | 129 | ||
| Granulomatous Lymphadenitis | 129 | ||
| Nonnecrotizing Granuloma | 129 | ||
| Necrotizing Granuloma | 130 | ||
| Tuberculosis. | 130 | ||
| Fungal Infection. | 130 | ||
| Suppurative Granuloma | 130 | ||
| Cat-Scratch Disease. | 130 | ||
| Kimura’s Disease | 131 | ||
| Toxoplasmic Lymphadenitis | 131 | ||
| Systemic Lupus Erythematosus | 132 | ||
| Kikuchi’s Lymphadenitis | 132 | ||
| Kawasaki’s Disease | 134 | ||
| Inflammatory Pseudotumor | 135 | ||
| Bacillary Angiomatosis | 135 | ||
| Diffuse Pattern | 135 | ||
| Infectious Mononucleosis | 135 | ||
| Cytomegalovirus Infection | 136 | ||
| Herpes Simplex Lymphadenitis | 137 | ||
| Dilantin-Associated Lymphadenopathy | 138 | ||
| References | 139.e1 | ||
| 10 The Normal Bone Marrow | 140 | ||
| Chapter Outline | 140 | ||
| Hematopoiesis | 142 | ||
| Erythropoiesis | 142 | ||
| Granulopoiesis | 142 | ||
| Megakaryocytes and Thrombopoiesis | 147 | ||
| Other Myeloid Cells | 148 | ||
| Cytologic Abnormalities in Myeloid Cells in Hematologically Normal Subjects | 149 | ||
| Bone Marrow Lymphoid Cells | 150 | ||
| Other Cells Present in Normal Bone Marrow | 150 | ||
| Normal Bone Marrow Components | 150 | ||
| Extraneous Cells and Tissues | 151 | ||
| Cytochemistry and Histochemistry | 151 | ||
| Cytochemistry | 151 | ||
| Histochemistry | 151 | ||
| Immunophenotyping | 153 | ||
| Conclusion | 153 | ||
| References | 153.e1 | ||
| 11 Evaluation of Anemia, Leukopenia, and Thrombocytopenia | 154 | ||
| Chapter Outline | 154 | ||
| Evaluation of Anemia | 154 | ||
| Microcytic Anemia | 155 | ||
| Iron Deficiency | 155 | ||
| Thalassemias | 158 | ||
| Normochromic Normocytic Anemia or Hypochromic Microcytic Anemia | 161 | ||
| Anemia of Chronic Disease | 161 | ||
| Sideroblastic Anemias | 161 | ||
| Normochromic Normocytic Anemia, Underproduction | 164 | ||
| Pure Red Cell Aplasia | 164 | ||
| Aplastic Anemia | 166 | ||
| Myelophthisic Anemias | 166 | ||
| Anemia of Chronic Renal Failure | 166 | ||
| Normochromic Normocytic Anemias, High Output | 166 | ||
| Posthemorrhagic Anemia | 166 | ||
| Hemolytic Anemias | 167 | ||
| Hemolysis Due to Intrinsic Red Cell Disorders. | 167 | ||
| Red Blood Cell Membrane Disorders. | 167 | ||
| Hereditary Spherocytosis. | 167 | ||
| Hereditary Elliptocytosis and Hereditary Pyropoikilocytosis. | 169 | ||
| Hereditary Stomatocytosis Syndromes. | 170 | ||
| Red Blood Cell Enzyme Defects. | 170 | ||
| Hemoglobinopathies. | 171 | ||
| Immune-Mediated Hemolytic Anemia | 172 | ||
| Autoimmune Hemolytic Anemias. | 172 | ||
| Drug-Induced Immune Hemolysis. | 173 | ||
| Hemolysis Due to Physicomechanical Disruption. | 173 | ||
| Infection-Associated Hemolytic Anemia. | 173 | ||
| Macrocytic Anemia | 173 | ||
| Megaloblastic Anemias | 174 | ||
| Constitutional Causes | 177 | ||
| Congenital Dyserythropoietic Anemias | 177 | ||
| Evaluation of Leukopenia | 178 | ||
| Neutropenia | 178 | ||
| Acquired Neutropenia in Infancy and Childhood | 179 | ||
| Neonatal Alloimmune Neutropenia. | 179 | ||
| Primary Autoimmune Neutropenia. | 179 | ||
| Acquired Neutropenia in Adults | 179 | ||
| Drug-Induced Neutropenia. | 180 | ||
| Secondary Autoimmune Neutropenia. | 180 | ||
| Nonimmune Chronic Idiopathic Neutropenia. | 180 | ||
| Infection-Related Neutropenia. | 180 | ||
| Nutritional Deficiency–Related Neutropenia. | 180 | ||
| Congenital Neutropenia | 180 | ||
| Severe Congenital Neutropenia. | 180 | ||
| Kostmann’s Neutropenia. | 181 | ||
| Cyclic Neutropenia. | 181 | ||
| Shwachman-Diamond Syndrome. | 182 | ||
| Chédiak-Higashi Syndrome. | 182 | ||
| Myelokathexis. | 182 | ||
| Dyskeratosis Congenita. | 182 | ||
| Lymphopenia | 182 | ||
| Therapeutic Agents | 183 | ||
| Congenital Disorders | 183 | ||
| Reactive Disorders | 183 | ||
| Autoimmune Disorders | 183 | ||
| Evaluation of Thrombocytopenia | 183 | ||
| Immune-Mediated Thrombocytopenic Purpura | 184 | ||
| Primary Immune Thrombocytopenia | 184 | ||
| Secondary Immune Thrombocytopenia | 187 | ||
| Heparin-Induced Thrombocytopenia | 187 | ||
| Infection-Associated Thrombocytopenia | 187 | ||
| Microangiopathic Processes | 188 | ||
| Splenic Sequestration | 188 | ||
| Constitutional Thrombocytopenia | 188 | ||
| Evaluation of Specific Bone Marrow Failure Syndromes | 189 | ||
| Paroxysmal Nocturnal Hemoglobinuria | 189 | ||
| Aplastic Anemia | 190 | ||
| Fanconi’s Anemia | 191 | ||
| Dyskeratosis Congenita | 192 | ||
| References | 193.e1 | ||
| 12 Bone Marrow Findings in Inflammatory, Infectious, and Metabolic Disorders | 194 | ||
| CHAPTER OUTLINE | 194 | ||
| Reactive Neutrophilia | 194 | ||
| Reactive Lymphocytosis | 194 | ||
| Reactive Eosinophilia | 195 | ||
| Reactive Basophilia | 197 | ||
| Reactive Monocytosis | 197 | ||
| Bone Marrow in Infectious Disorders | 197 | ||
| Bacterial Infection | 197 | ||
| Rickettsial Infection | 198 | ||
| Parasitic Infection | 199 | ||
| Viral Infection | 199 | ||
| Cytomegalovirus | 199 | ||
| Epstein-Barr Virus | 200 | ||
| Hepatitis | 201 | ||
| Hantavirus | 201 | ||
| Parvovirus B19 and Human Immunodeficiency Virus | 201 | ||
| Fungal Infection | 201 | ||
| Bone Marrow Necrosis | 201 | ||
| Fever of Unknown Origin | 202 | ||
| Bone Marrow in Noninfectious Systemic and Inflammatory Disorders | 203 | ||
| Noninfectious Granuloma | 203 | ||
| Connective Tissue Disease | 203 | ||
| Sarcoidosis | 205 | ||
| Alcohol Abuse | 206 | ||
| Hepatic Disease | 206 | ||
| Renal Disease | 206 | ||
| Hypothyroidism | 207 | ||
| Hyperthyroidism | 207 | ||
| Conclusion | 207 | ||
| References | 208.e1 | ||
| III Lymphoid Neoplasms | 209 | ||
| 13 Principles of Classification of Lymphoid Neoplasms | 211 | ||
| Chapter Outline | 211 | ||
| Historical Background | 211 | ||
| Emerging Concepts: 2008 World Health Organization Classification | 214 | ||
| Early Events in Lymphoid Neoplasia: Borders of Malignancy | 215 | ||
| Age as a Feature of Disease Definition | 216 | ||
| Aggressive B-Cell Lymphomas and Borderline Malignancies | 217 | ||
| Follicular Lymphoma: Grading and Genetic Heterogeneity | 218 | ||
| Peripheral T-Cell Lymphomas | 218 | ||
| Precursor Lymphoid Neoplasms | 219 | ||
| Histiocytic and Dendritic Cell Neoplasms | 219 | ||
| Conclusion | 220 | ||
| References | 220.e1 | ||
| 1 Mature B-Cell Neoplasms | 221 | ||
| 14 Mature B-Cell Neoplasms: | 221 | ||
| Chapter Outline | 221 | ||
| B-Cell Chronic Lymphocytic Leukemia–Small Lymphocytic Lymphoma | 221 | ||
| Definition of Disease | 221 | ||
| Epidemiology and Incidence | 222 | ||
| Clinical Features | 222 | ||
| Morphology | 222 | ||
| Lymph Node | 222 | ||
| Spleen | 223 | ||
| Blood | 223 | ||
| Bone Marrow | 224 | ||
| Other Organs | 226 | ||
| Morphologic Variants | 227 | ||
| Atypical Chronic Lymphocytic Leukemia | 227 | ||
| Plasmacytoid Differentiation | 227 | ||
| Reed-Sternberg Cells | 228 | ||
| Mu Heavy-Chain Disease | 228 | ||
| Immunophenotype | 228 | ||
| Prognostic Markers: CD38 and ZAP-70 | 229 | ||
| Genetic and Molecular Features | 230 | ||
| Deletion of 13q14 | 231 | ||
| Trisomy 12 | 232 | ||
| Deletion of 11q22-q23 | 232 | ||
| Deletion of 17p13 | 232 | ||
| Postulated Cell of Origin and Normal Counterpart Cell | 232 | ||
| Clinical Course, Prognostic Factors, and Transformation | 232 | ||
| Differential Diagnosis | 233 | ||
| B-Cell Prolymphocytic Leukemia | 236 | ||
| Definition of Disease | 236 | ||
| Epidemiology and Incidence | 237 | ||
| Clinical Features | 237 | ||
| Morphology | 237 | ||
| Peripheral Blood and Bone Marrow | 237 | ||
| Spleen | 237 | ||
| Lymph Node | 237 | ||
| Other Tissue Sites | 237 | ||
| Immunophenotype | 238 | ||
| Cytogenetic and Molecular Genetic Features | 238 | ||
| Cell of Origin | 238 | ||
| Clinical Course | 239 | ||
| Differential Diagnosis | 239 | ||
| Lymphoplasmacytic Lymphoma and Waldenström’s Macroglobulinemia | 240 | ||
| Definition of Disease | 240 | ||
| Epidemiology and Incidence | 241 | ||
| Clinical Features | 241 | ||
| Morphology | 241 | ||
| Peripheral Blood and Bone Marrow | 241 | ||
| Lymph Nodes | 242 | ||
| Spleen, Liver, and Other Tissue Sites | 242 | ||
| Immunophenotype | 243 | ||
| Cytogenetic and Molecular Genetic Features | 243 | ||
| Cell of Origin | 244 | ||
| Clinical Course | 245 | ||
| Differential Diagnosis | 245 | ||
| References | 246.e1 | ||
| 15 Hairy Cell Leukemia | 247 | ||
| Chapter Outline | 247 | ||
| Definition of Disease and Nomenclature | 247 | ||
| Epidemiology | 247 | ||
| Etiology | 247 | ||
| Clinical Features | 247 | ||
| Symptoms and Signs | 247 | ||
| Imaging Studies | 248 | ||
| Diagnostic Procedures | 248 | ||
| Staging | 248 | ||
| Morphology | 248 | ||
| Cell Morphology on Smear Preparations | 248 | ||
| Cell Morphology in Bone Marrow Sections | 250 | ||
| Spleen and Other Organs | 250 | ||
| Variants | 250 | ||
| Phenotype | 251 | ||
| Flow Cytometry | 251 | ||
| Cytochemistry | 253 | ||
| Immunohistochemistry | 253 | ||
| Genetics and Molecular Findings | 253 | ||
| Postulated Cell of Origin and Normal Counterpart | 253 | ||
| Clinical Course and Treatment | 254 | ||
| Prognostic and Predictive Factors | 254 | ||
| Differential Diagnosis | 254 | ||
| References | 255.e1 | ||
| 16 Splenic Marginal Zone Lymphoma | 256 | ||
| Chapter Outline | 256 | ||
| Definition | 256 | ||
| Epidemiology | 256 | ||
| Etiology | 256 | ||
| Clinical Features | 258 | ||
| Morphology | 258 | ||
| Immunophenotype | 258 | ||
| Genetics | 258 | ||
| Genetic Abnormalities | 258 | ||
| Antigen Receptor Genes | 259 | ||
| Gene Expression Profiling | 259 | ||
| Cell of Origin | 261 | ||
| Clinical Course | 261 | ||
| Differential Diagnosis | 261 | ||
| Other Splenic B-Cell Lymphomas | 263 | ||
| Splenic Diffuse Red Pulp Small B-Cell Lymphoma | 263 | ||
| Hairy Cell Leukemia Variant | 263 | ||
| References | 266.e1 | ||
| 17 Follicular Lymphoma | 267 | ||
| Chapter Outline | 267 | ||
| Definition | 267 | ||
| Epidemiology | 268 | ||
| Clinical Features | 269 | ||
| Sites of Involvement | 269 | ||
| Clinical Evaluation and Staging | 269 | ||
| Morphologic Features | 269 | ||
| Cellular Composition | 269 | ||
| Grading | 272 | ||
| Pattern | 272 | ||
| Diffuse Areas in Follicular Lymphoma | 275 | ||
| Diffuse Follicular Lymphoma | 275 | ||
| Partial Nodal Involvement | 275 | ||
| Morphology in Sites Other Than Lymph Nodes | 275 | ||
| Spleen | 275 | ||
| Bone Marrow | 276 | ||
| Peripheral Blood | 276 | ||
| Histologic Transformation | 276 | ||
| Immunophenotype | 276 | ||
| Genetic Features | 281 | ||
| Antigen Receptor Genes | 281 | ||
| Cytogenetic Abnormalities and Oncogenes | 281 | ||
| Cytogenetic Abnormalities | 281 | ||
| Oncogenes | 282 | ||
| BCL2. | 282 | ||
| BCL6. | 282 | ||
| MYC. | 282 | ||
| Gene Expression Profiles | 282 | ||
| Postulated Normal Counterpart | 283 | ||
| Etiology | 283 | ||
| Clinical Course | 283 | ||
| Natural History | 283 | ||
| Treatment | 283 | ||
| Prognosis and Predictive Factors | 283 | ||
| Clinical Factors | 283 | ||
| Histologic Grade | 284 | ||
| Diffuse Areas | 284 | ||
| Histologic Transformation | 284 | ||
| Variants | 284 | ||
| Pediatric Follicular Lymphoma | 284 | ||
| Primary Intestinal Follicular Lymphoma | 284 | ||
| Intrafollicular Neoplasia (in Situ Follicular Lymphoma) | 284 | ||
| Differential Diagnosis | 286 | ||
| Follicular Hyperplasia | 286 | ||
| Morphologic Criteria | 286 | ||
| Pattern. | 286 | ||
| Cytology. | 287 | ||
| Immunophenotyping. | 287 | ||
| Molecular Genetic Analysis | 288 | ||
| Other Lymphomas | 288 | ||
| Small B-Cell Lymphomas | 288 | ||
| Morphologic Features. | 288 | ||
| Immunophenotype. | 289 | ||
| Genetic Analysis. | 289 | ||
| Hodgkin’s Lymphoma | 289 | ||
| Conclusion | 290 | ||
| References | 290.e1 | ||
| 18 Extranodal Marginal Zone Lymphoma: | 291 | ||
| Chapter Outline | 291 | ||
| Histology and Immunology of Mucosa-Associated Lymphoid Tissue | 291 | ||
| Peyer’s Patches | 291 | ||
| Definition of Malt Lymphoma | 292 | ||
| Epidemiology | 293 | ||
| Etiology | 293 | ||
| Infectious Agents | 293 | ||
| Helicobacter pylori and Gastric MALT Lymphoma | 293 | ||
| Campylobacter jejuni and Immunoproliferative Small Intestinal Disease | 294 | ||
| Borrelia burgdorferi and Cutaneous MALT Lymphoma | 294 | ||
| Chlamydia psittaci and Ocular Adnexal MALT Lymphoma | 295 | ||
| Establishing an Etiologic Link | 295 | ||
| Histopathology of Acquired Malt | 295 | ||
| Salivary Gland Acquired MALT (Lymphoepithelial Sialadenitis ) | 295 | ||
| Helicobacter pylori Gastritis | 295 | ||
| Pathology of Malt Lymphoma | 297 | ||
| Macroscopic Appearance | 297 | ||
| Histopathology | 297 | ||
| Morphology of Gastric MALT Lymphoma Following Eradication of Helicobacter pylori | 299 | ||
| Dissemination | 300 | ||
| Immunohistochemistry | 301 | ||
| Genetic Features of Malt Lymphoma | 301 | ||
| Antigen Receptor Genes | 301 | ||
| Genetic Abnormalities | 302 | ||
| Postulated Cell of Origin | 303 | ||
| Clinical Course | 303 | ||
| Differential Diagnosis | 304 | ||
| Reactive versus Neoplastic MALT | 304 | ||
| MALT versus Other Small B-Cell Lymphomas | 304 | ||
| References | 305.e1 | ||
| 19 Primary Cutaneous B-Cell Lymphoma | 306 | ||
| Chapter Outline | 306 | ||
| Clinical Features and Clinical Presentation | 306 | ||
| Epidemiology | 307 | ||
| Histopathology | 307 | ||
| Immunophenotype | 307 | ||
| Gene Rearrangement | 307 | ||
| Treatment and Prognosis | 307 | ||
| Classification of Primary Cutaneous Lymphoma | 308 | ||
| B-Cell Lymphomas of the Skin | 308 | ||
| Primary Cutaneous Marginal Zone Lymphoma | 308 | ||
| Definition | 308 | ||
| Epidemiology | 308 | ||
| Etiology | 309 | ||
| Clinical Features | 309 | ||
| Histopathology | 309 | ||
| Immunophenotype | 309 | ||
| Genetics | 309 | ||
| Postulated Normal Counterpart | 311 | ||
| Clinical Course | 311 | ||
| Differential Diagnosis | 311 | ||
| Primary Cutaneous Follicle Center Lymphoma | 312 | ||
| Definition | 312 | ||
| Epidemiology | 312 | ||
| Etiology | 312 | ||
| Clinical Features | 312 | ||
| Histopathology | 312 | ||
| Grading | 313 | ||
| Immunophenotype | 313 | ||
| Genetics | 314 | ||
| Postulated Normal Counterpart | 314 | ||
| Clinical Course | 314 | ||
| Differential Diagnosis | 314 | ||
| Diffuse Large B-Cell Lymphoma–Leg Type | 314 | ||
| Definition | 314 | ||
| Epidemiology | 314 | ||
| Clinical Features | 315 | ||
| Histopathology | 315 | ||
| Immunophenotype | 315 | ||
| Genetics and Postulated Normal Counterpart | 315 | ||
| Clinical Course | 315 | ||
| Differential Diagnosis | 315 | ||
| Diffuse Large B-Cell Lymphoma–Other | 315 | ||
| Plasmacytoma | 317 | ||
| Plasmablastic Lymphoma | 317 | ||
| B-Lymphoblastic Leukemia/Lymphoma | 317 | ||
| Reactive Lymphoid Infiltrates that may Mimic Lymphoma | 317 | ||
| References | 318.e1 | ||
| 20 Nodal Marginal Zone Lymphoma | 319 | ||
| Chapter Outline | 319 | ||
| Definition | 319 | ||
| Epidemiology, Etiology, and Cofactors | 319 | ||
| Clinical Features | 319 | ||
| Morphology | 320 | ||
| Cytologic Features | 320 | ||
| Architectural Features | 320 | ||
| Other Anatomic Sites | 324 | ||
| Grading | 324 | ||
| Immunophenotype | 324 | ||
| Genetics and Molecular Findings | 324 | ||
| Postulated Cell of Origin | 326 | ||
| Clinical Course and Prognostic Factors | 326 | ||
| Pediatric Nodal Marginal Zone Lymphoma | 326 | ||
| Morphology and Immunophenotype | 326 | ||
| Genetic Features | 327 | ||
| Clinical Features | 327 | ||
| Differential Diagnosis | 327 | ||
| Extranodal Marginal Zone Lymphoma | 327 | ||
| Lymphoplasmacytic Lymphoma | 328 | ||
| Marginal Zone Hyperplasia and Related Reactive Conditions | 329 | ||
| Monocytoid B-Cell Hyperplasia | 329 | ||
| Follicular Lymphoma with Marginal Zone Differentiation | 329 | ||
| Chronic Lymphocytic Leukemia–Small Lymphocytic Lymphoma with a Parafollicular Pattern | 329 | ||
| Splenic Marginal Zone Lymphoma | 329 | ||
| References | 332.e1 | ||
| 21 Mantle Cell Lymphoma | 333 | ||
| Chapter Outline | 333 | ||
| Definition | 333 | ||
| Epidemiology and Clinical Manifestations | 334 | ||
| Postulated Cell of Origin | 334 | ||
| Morphology | 335 | ||
| Architectural Patterns | 335 | ||
| Cytologic Variants | 335 | ||
| Bone Marrow and Peripheral Blood | 336 | ||
| Spleen | 337 | ||
| Gastrointestinal Tract | 337 | ||
| Histologic Progression | 339 | ||
| Composite Mantle Cell Lymphoma and Other Lymphoproliferative Disorders | 339 | ||
| Immunophenotype | 339 | ||
| Cytogenetic Findings | 340 | ||
| Molecular Characteristics | 341 | ||
| Translocation (11;14) | 341 | ||
| Cyclin D1 Messenger RNA Transcripts and Polymorphism | 342 | ||
| Cyclin D1 Oncogenic Mechanisms | 342 | ||
| Altered Molecular Pathways | 343 | ||
| Cell Cycle Deregulation | 343 | ||
| DNA Damage Response Genes | 344 | ||
| Cell Survival Pathways | 344 | ||
| Cyclin D1–Negative Mantle Cell Lymphoma | 344 | ||
| Clinical Course | 344 | ||
| Prognostic Parameters | 345 | ||
| Proliferative Activity | 345 | ||
| Architectural Growth Pattern | 345 | ||
| Cytologic Variants | 345 | ||
| Genetic Aberrations | 345 | ||
| Molecular Alterations | 346 | ||
| Clinical Parameters | 346 | ||
| Therapy | 346 | ||
| Differential Diagnosis | 347 | ||
| Benign Disorders | 347 | ||
| Cyclin D1–Negative Mantle Cell Lymphoma | 347 | ||
| Atypical Leukemic Lymphoid Neoplasms | 347 | ||
| Chronic Lymphocytic Leukemia–Small Lymphocytic Lymphoma | 347 | ||
| Follicular Lymphoma | 348 | ||
| Marginal Zone Lymphoma | 348 | ||
| Diffuse Large B-Cell Lymphoma | 348 | ||
| Acute Leukemias | 348 | ||
| References | 348.e1 | ||
| 22 Diffuse Large B-Cell Lymphoma | 349 | ||
| Chapter Outline | 349 | ||
| Definition | 349 | ||
| Epidemiology | 349 | ||
| Etiology | 350 | ||
| Clinical Features | 350 | ||
| Morphology | 350 | ||
| Immunophenotype | 354 | ||
| Genetics | 356 | ||
| Postulated Cell of Origin | 359 | ||
| Clinical Course | 359 | ||
| Prognostic Factors | 359 | ||
| Clinical | 359 | ||
| Morphologic | 360 | ||
| Immunohistochemical | 360 | ||
| Molecular | 360 | ||
| Differential Diagnosis | 361 | ||
| Subtypes and Distinct Disease Entities | 364 | ||
| De Novo CD5+ Diffuse Large B-Cell Lymphoma | 364 | ||
| Definition | 364 | ||
| Epidemiology | 364 | ||
| Clinical Features | 364 | ||
| Morphology | 364 | ||
| Immunophenotype | 364 | ||
| Genetics | 364 | ||
| Postulated Cell of Origin | 366 | ||
| Clinical Course | 366 | ||
| Differential Diagnosis | 366 | ||
| T-Cell/Histiocyte-Rich Large B-Cell Lymphoma | 366 | ||
| Definition | 366 | ||
| Epidemiology | 366 | ||
| Etiology | 366 | ||
| Clinical Features | 366 | ||
| Morphology | 366 | ||
| Immunophenotype | 367 | ||
| Genetics | 367 | ||
| Postulated Cell of Origin | 367 | ||
| Clinical Course | 367 | ||
| Differential Diagnosis | 367 | ||
| EBV+ Diffuse Large B-Cell Lymphoma of the Elderly | 369 | ||
| Definition | 369 | ||
| Epidemiology and Etiology | 369 | ||
| Clinical Features | 369 | ||
| Pathology | 370 | ||
| Primary Mediastinal (Thymic) Large B-Cell Lymphoma | 370 | ||
| Definition | 370 | ||
| Epidemiology | 370 | ||
| Etiology | 370 | ||
| Clinical Features | 370 | ||
| Morphology | 370 | ||
| Immunophenotype | 370 | ||
| Genetics | 370 | ||
| Postulated Cell of Origin | 372 | ||
| Clinical Course | 372 | ||
| Differential Diagnosis | 373 | ||
| Intravascular Large B-Cell Lymphoma | 374 | ||
| Definition | 374 | ||
| Epidemiology | 374 | ||
| Etiology | 374 | ||
| Clinical Features | 374 | ||
| Morphology | 375 | ||
| Immunophenotype | 375 | ||
| Genetics | 376 | ||
| Postulated Cell of Origin | 376 | ||
| Clinical Course | 376 | ||
| Differential Diagnosis | 376 | ||
| Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation | 377 | ||
| Definition | 377 | ||
| Pyothorax-Associated Lymphoma | 377 | ||
| Epidemiology. | 377 | ||
| Etiology. | 377 | ||
| Clinical Features. | 377 | ||
| Morphology. | 377 | ||
| Immunophenotype. | 377 | ||
| Genetics. | 377 | ||
| Differential Diagnosis. | 377 | ||
| Other Inflammation-Associated Lymphomas | 377 | ||
| ALK+ Large B-Cell Lymphoma | 378 | ||
| IV Myeloid Neoplasms | 647 | ||
| 43 Principles of Classification of Myeloid Neoplasms | 649 | ||
| Chapter Outline | 649 | ||
| Evaluation of Myeloid Neoplasms | 650 | ||
| Who Classification | 652 | ||
| Myeloproliferative Neoplasms | 654 | ||
| Myeloid and Lymphoid Neoplasms with Eosinophilia and Abnormalities of PDGFRA, PDGFRB, and FGFR1 | 654 | ||
| Myelodysplastic/Myeloproliferative Neoplasms | 654 | ||
| Myelodysplastic Syndromes | 654 | ||
| Acute Myeloid Leukemia | 655 | ||
| Acute Leukemias of Ambiguous Lineage | 655 | ||
| Conclusion | 655 | ||
| References | 655.e1 | ||
| 44 The Myelodysplastic Syndromes | 656 | ||
| Chapter Outline | 656 | ||
| Incidence | 657 | ||
| Clinical Features | 657 | ||
| Laboratory Features | 657 | ||
| Peripheral Blood Parameters | 657 | ||
| Microscopic Features in Peripheral Blood and Bone Marrow | 657 | ||
| Extramedullary Manifestations | 658 | ||
| Cytogenetic Abnormalities | 658 | ||
| Other Biologic Abnormalities and Assessments | 659 | ||
| Clonality | 659 | ||
| Loss of Function in Hematopoietic Cells | 659 | ||
| Flow Cytometric Analysis | 660 | ||
| Apoptosis and Cell Cycle Analysis | 661 | ||
| Molecular Biologic Abnormalities | 662 | ||
| Diagnosis | 662 | ||
| Classification | 663 | ||
| Myelodysplastic Syndrome–Related Disease versus De Novo Acute Myeloid Leukemia | 663 | ||
| Stages of Myelodysplastic Syndrome | 663 | ||
| Low-Grade versus High-Grade Myelodysplastic Syndrome | 663 | ||
| High-Grade Myelodysplastic Syndrome versus MDS-Related Acute Myeloid Leukemia | 663 | ||
| World Health Organization Classification | 663 | ||
| Refractory Cytopenia with Unilineage Dysplasia | 664 | ||
| Refractory Anemia with Ring Sideroblasts | 665 | ||
| Refractory Cytopenia with Multilineage Dysplasia | 665 | ||
| Refractory Anemia with Excess Blasts | 665 | ||
| Myelodysplastic Syndrome Associated with Isolated 5q– Syndrome | 665 | ||
| Myelodysplastic Syndrome, Unclassifiable | 666 | ||
| Myelodysplastic Syndrome in Childhood | 666 | ||
| Therapy-Related Myelodysplastic Syndrome | 666 | ||
| Additional Considerations | 666 | ||
| Hypocellular Myelodysplastic Syndrome | 666 | ||
| Myelodysplastic Syndrome with 3q26 Abnormalities | 667 | ||
| Myelodysplastic Syndrome with 17p Abnormalities | 667 | ||
| Fibrosis | 667 | ||
| Prognostic Scoring Systems | 667 | ||
| Differential Diagnosis | 667 | ||
| Megaloblastic Anemias | 667 | ||
| Chemotherapy-Induced Dysplasia and Cytopenias versus Therapy-Related Myelodysplastic Syndrome | 667 | ||
| Marrow Response after Chemotherapy in MDS-Related Acute Myeloid Leukemia | 668 | ||
| Marrow Recovery after Stem Cell Transplantation (or Other Aplastic Events) | 668 | ||
| De Novo Acute Myeloid Leukemia with Low Blast Count | 668 | ||
| Marrow Dysplasia in AIDS | 668 | ||
| Congenital Dyserythropoietic Anemias | 668 | ||
| Non–Myelodysplastic Syndrome Sideroblastic Anemias | 669 | ||
| Copper Deficiency and Zinc Toxicity | 669 | ||
| Chronic Viral Infections | 669 | ||
| Cytogenetic Abnormalities Mimicking Myelodysplastic Syndrome | 669 | ||
| Reticulin Fibrosis in Myelodysplastic Syndrome versus Primary Myelofibrosis | 669 | ||
| Reticulin Fibrosis in Myelodysplastic Syndrome versus Acute Megakaryoblastic Leukemia | 670 | ||
| Leukemoid Reaction in Low-Grade Myelodysplastic Syndrome versus Chronic Myelomonocytic Leukemia | 670 | ||
| Aplastic Anemia versus Hypocellular Myelodysplastic Syndrome | 670 | ||
| Paroxysmal Nocturnal Hemoglobinuria | 670 | ||
| Arsenic Exposure | 670 | ||
| Causative Agents | 670 | ||
| Pathogenesis | 671 | ||
| Conclusion | 671 | ||
| References | 671.e1 | ||
| 45 Acute Myeloid Leukemia | 672 | ||
| Chapter Outline | 672 | ||
| Epidemiology | 673 | ||
| Etiology | 673 | ||
| Clinical Features | 673 | ||
| Morphology | 673 | ||
| Cytochemistry | 673 | ||
| Immunophenotype | 673 | ||
| Genetics | 674 | ||
| Prognosis | 674 | ||
| Acute Myeloid Leukemia with Recurrent Genetic Abnormalities | 674 | ||
| Acute Myeloid Leukemia with t(8;21)(q22;q22) (RUNX1-RUNX1T1) | 674 | ||
| Acute Myeloid Leukemia with inv(16)(p13q22) or t(16;16)(p13;q22) (CBFB-MYH11) | 675 | ||
| Acute Promyelocytic Leukemia (APL) with t(15;17)(q22;q12) (PML-RARA) | 676 | ||
| Acute Promyelocytic Leukemia with Variant RARA Translocations | 678 | ||
| Acute Myeloid Leukemia with t(9;11)(p22;q23) (MLLT3-MLL) | 678 | ||
| Acute Myeloid Leukemia with t(6;9)(p23;q34) (DEK-NUP214) | 680 | ||
| Acute Myeloid Leukemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2) (RPN1-EVI1) | 681 | ||
| Acute Myeloid Leukemia (Megakaryoblastic) with t(1;22)(p13;q13) (RBM15-MKL1) | 682 | ||
| Other Acute Myeloid Leukemias with Recurrent Genetic Abnormalities | 683 | ||
| Acute Myeloid Leukemia with Gene Mutations | 683 | ||
| Acute Myeloid Leukemia with Mutated NPM1 | 684 | ||
| Acute Myeloid Leukemia with Mutated CEBPA | 685 | ||
| Acute Myeloid Leukemia with Myelodysplasia-Related Changes | 685 | ||
| Therapy-Related Myeloid Neoplasms | 687 | ||
| Acute Myeloid Leukemia, Not Otherwise Specified | 688 | ||
| Acute Myeloid Leukemia with Minimal Differentiation | 689 | ||
| Acute Myeloid Leukemia without Maturation | 689 | ||
| Acute Myeloid Leukemia with Maturation | 689 | ||
| Acute Myelomonocytic Leukemia | 689 | ||
| Acute Monoblastic and Monocytic Leukemias | 689 | ||
| Acute Erythroid Leukemia | 690 | ||
| Acute Megakaryoblastic Leukemia | 691 | ||
| Acute Basophilic Leukemia | 691 | ||
| Acute Panmyelosis with Myelofibrosis | 692 | ||
| Myeloid Proliferations of Down Syndrome | 692 | ||
| Transient Abnormal Myelopoiesis | 692 | ||
| Myeloid Leukemia Associated with Down Syndrome | 693 | ||
| Myeloid Sarcoma | 694 | ||
| Integrated Approach to the Diagnosis of Acute Myeloid Leukemia | 696 | ||
| References | 697.e1 | ||
| 46 Myeloproliferative Neoplasms | 698 | ||
| Chapter Outline | 698 | ||
| Definition | 698 | ||
| Etiology and Pathogenesis | 699 | ||
| Chronic Myelogenous Leukemia, BCR-ABL1 Positive | 703 | ||
| Diagnosis | 703 | ||
| Clinical Findings | 703 | ||
| Laboratory Findings | 703 | ||
| Peripheral Blood | 703 | ||
| Bone Marrow | 704 | ||
| Extramedullary Tissues | 705 | ||
| Cytogenetics and Genetics | 705 | ||
| Disease Progression and Prognosis | 707 | ||
| Accelerated Phase | 708 | ||
| Blast Phase | 708 | ||
| Appearance of BCR-ABL1–Negative Clonal Myeloid Proliferations | 711 | ||
| Therapy and Disease Monitoring | 711 | ||
| Differential Diagnosis | 711 | ||
| Chronic Phase | 711 | ||
| Transformed Stages | 713 | ||
| Chronic Neutrophilic Leukemia | 713 | ||
| Diagnosis | 714 | ||
| Clinical Findings | 714 | ||
| Laboratory Findings | 714 | ||
| Peripheral Blood | 714 | ||
| Bone Marrow | 715 | ||
| Extramedullary Tissues | 715 | ||
| Genetics | 715 | ||
| Disease Progression and Prognosis | 715 | ||
| Differential Diagnosis | 715 | ||
| Polycythemia Vera | 715 | ||
| Diagnosis | 716 | ||
| Clinical Findings, Polycythemic Phase | 716 | ||
| Laboratory Findings, Polycythemic Phase | 717 | ||
| Peripheral Blood | 717 | ||
| Bone Marrow | 717 | ||
| Extramedullary Tissues | 718 | ||
| Genetics | 718 | ||
| Other Laboratory Findings | 718 | ||
| Disease Progression and Prognosis | 719 | ||
| Post-polycythemic Myeloid Metaplasia | 719 | ||
| Acute Leukemia/Myelodysplastic Phase | 719 | ||
| Differential Diagnosis | 720 | ||
| Primary Polycythemia, Acquired and Congenital | 720 | ||
| Secondary Polycythemia, Acquired and Congenital | 720 | ||
| Primary Myelofibrosis | 721 | ||
| Diagnosis | 721 | ||
| Clinical Findings | 721 | ||
| Laboratory Findings | 722 | ||
| Peripheral Blood | 722 | ||
| Bone Marrow | 723 | ||
| Extramedullary Tissues | 723 | ||
| Genetics | 727 | ||
| Other Laboratory Findings | 727 | ||
| Disease Progression and Prognosis | 727 | ||
| Differential Diagnosis | 727 | ||
| Essential Thrombocythemia | 728 | ||
| Diagnosis | 729 | ||
| Clinical Findings | 729 | ||
| Laboratory Findings | 729 | ||
| Peripheral Blood | 729 | ||
| Bone Marrow | 730 | ||
| Extramedullary Tissues | 731 | ||
| Genetics | 731 | ||
| Other Laboratory Findings | 731 | ||
| Disease Progression and Prognosis | 731 | ||
| Differential Diagnosis | 731 | ||
| Other Myeloid Neoplasms Associated with Thrombocytosis | 731 | ||
| Myeloproliferative Neoplasms, Unclassifiable | 731 | ||
| References | 732.e1 | ||
| 47 Myelodysplastic/Myeloproliferative Neoplasms | 733 | ||
| Chapter Outline | 733 | ||
| Etiology and Pathogenesis | 733 | ||
| Chronic Myelomonocytic Leukemia | 734 | ||
| Clinical Findings | 735 | ||
| Laboratory Findings | 736 | ||
| Blood | 736 | ||
| Bone Marrow | 739 | ||
| Extramedullary Tissues | 739 | ||
| Immunophenotype | 739 | ||
| Cytogenetics and Genetics | 743 | ||
| Other Laboratory Findings | 744 | ||
| Differential Diagnosis | 744 | ||
| Reactive Monocytosis | 745 | ||
| Acute Myelomonocytic and Acute Monocytic Leukemia | 745 | ||
| Chronic Myelogenous Leukemia, BCR-ABL1 Positive | 745 | ||
| Myeloid Neoplasms Associated with Eosinophilia and Rearrangements of PDGFRB | 745 | ||
| Atypical Chronic Myeloid Leukemia | 745 | ||
| BCR-ABL1 Negative Myeloproliferative Neoplasms Associated with Monocytosis | 746 | ||
| Myelodysplastic Syndrome | 746 | ||
| Prognosis, Prognostic Factors, and Progression | 746 | ||
| Atypical Chronic Myeloid LeukEmia, Bcr-Abl1 Negative | 747 | ||
| Clinical Findings | 747 | ||
| Laboratory Findings | 747 | ||
| Blood | 747 | ||
| Bone Marrow | 748 | ||
| Extramedullary Tissues | 749 | ||
| Immunophenotype | 749 | ||
| Cytogenetics and Genetics | 749 | ||
| Differential Diagnosis | 749 | ||
| Chronic Myelomonocytic Leukemia | 749 | ||
| Chronic Myelogenous Leukemia, BCR-ABL1 Positive | 749 | ||
| Myelodysplastic Syndrome | 749 | ||
| Prognosis and Prognostic Factors | 749 | ||
| Juvenile Myelomonocytic Leukemia | 750 | ||
| Clinical Findings | 750 | ||
| Laboratory Findings | 750 | ||
| Blood | 750 | ||
| Bone Marrow | 750 | ||
| Extramedullary Tissues | 752 | ||
| Immunophenotype | 752 | ||
| Cytogenetics and Genetics | 752 | ||
| Other Laboratory Findings | 752 | ||
| Differential Diagnosis | 752 | ||
| Infection | 752 | ||
| Other Myeloid Diseases | 754 | ||
| Prognosis and Outcome | 754 | ||
| Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable | 754 | ||
| Refractory Anemia with Ring Sideroblasts Associated with Marked Thrombocytosis | 755 | ||
| Clinical Findings | 755 | ||
| Laboratory Findings | 755 | ||
| Blood. | 755 | ||
| Bone Marrow. | 755 | ||
| Immunophenotype. | 755 | ||
| Cytogenetics and Genetics. | 755 | ||
| Differential Diagnosis | 755 | ||
| Refractory Anemia with Ring Sideroblasts. | 755 | ||
| Myeloproliferative Neoplasm with Ring Sideroblasts. | 755 | ||
| Prognosis and Prognostic Features | 755 | ||
| References | 756.e1 | ||
| 48 Mastocytosis | 757 | ||
| Chapter Outline | 757 | ||
| Mast Cells and Basophils | 757 | ||
| Definition | 758 | ||
| General Aspects | 759 | ||
| Epidemiology and Etiology | 759 | ||
| Postulated Cell of Origin and Normal Counterpart | 759 | ||
| Genetics and Molecular Findings | 759 | ||
| Cytologic and Histologic Diagnosis | 760 | ||
| Histology | 761 | ||
| Immunophenotype | 762 | ||
| Histopathologic Findings | 763 | ||
| Bone Marrow | 764 | ||
| Spleen | 764 | ||
| Liver | 764 | ||
| Lymph Nodes | 765 | ||
| Gastrointestinal Tract | 765 | ||
| Skin | 767 | ||
| Differential Diagnosis | 767 | ||
| Classification | 768 | ||
| Cutaneous Mastocytosis | 769 | ||
| Definition | 769 | ||
| Epidemiology | 769 | ||
| Clinical Features | 770 | ||
| Morphology | 770 | ||
| Immunophenotype | 771 | ||
| Postulated Cell of Origin | 771 | ||
| Clinical Course | 771 | ||
| Differential Diagnosis | 771 | ||
| Indolent Systemic Mastocytosis | 771 | ||
| Definition | 771 | ||
| Epidemiology | 771 | ||
| Clinical Features | 771 | ||
| Morphology | 771 | ||
| Immunophenotype | 772 | ||
| Postulated Cell of Origin | 772 | ||
| Clinical Course | 772 | ||
| Provisional Subvariants and Differential Diagnosis | 772 | ||
| Systemic Mastocytosis with an Associated Clonal Hematologic Non–Mast Cell Lineage Disorder | 772 | ||
| Definition | 772 | ||
| Epidemiology | 772 | ||
| Clinical Features | 772 | ||
| Morphology | 772 | ||
| V Histiocytic Proliferations | 799 | ||
| 51 Nonneoplastic Histiocytic Proliferations of Lymph Nodes and Bone Marrow | 801 | ||
| Chapter Outline | 801 | ||
| Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease) | 801 | ||
| Definition | 801 | ||
| Epidemiology | 801 | ||
| Etiology | 801 | ||
| Clinical Features | 801 | ||
| Morphology | 802 | ||
| Immunophenotype | 802 | ||
| Clinical Course | 802 | ||
| Differential Diagnosis | 803 | ||
| Hemophagocytic Syndromes | 803 | ||
| Familial (Primary) Hemophagoctyic Lymphohistiocytosis | 803 | ||
| Definition | 803 | ||
| Epidemiology | 803 | ||
| Etiology | 803 | ||
| Clinical Features | 803 | ||
| Morphology | 804 | ||
| Immunophenotype | 804 | ||
| Genetics | 804 | ||
| Clinical Course | 805 | ||
| Secondary Hemophagocytic Syndromes | 805 | ||
| Definition | 805 | ||
| Epidemiology | 805 | ||
| Etiology | 805 | ||
| Clinical Features | 805 | ||
| Morphology | 805 | ||
| Immunophenotype | 805 | ||
| Genetics | 806 | ||
| Clinical Course | 806 | ||
| Differential Diagnosis | 806 | ||
| Storage Disorders | 807 | ||
| Niemann-Pick Disease | 807 | ||
| Definition | 807 | ||
| Etiology | 807 | ||
| Clinical Features | 807 | ||
| Morphology | 807 | ||
| Differential Diagnosis | 809 | ||
| Gaucher’s Disease | 809 | ||
| Definition | 809 | ||
| Etiology | 809 | ||
| Clinical Features | 809 | ||
| Morphology | 809 | ||
| Differential Diagnosis | 810 | ||
| Tangier Disease | 810 | ||
| Definition | 810 | ||
| Etiology | 810 | ||
| Clinical Features | 810 | ||
| Morphology | 810 | ||
| References | 810.e1 | ||
| 52 Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma | 811 | ||
| Chapter Outline | 811 | ||
| Langerhans Cell Histiocytosis | 811 | ||
| Definition | 811 | ||
| Epidemiology and Etiology | 811 | ||
| Clinical Features | 811 | ||
| Morphology | 812 | ||
| Grading | 812 | ||
| Immunophenotype | 812 | ||
| Congenital Langerhans Cell Disease | 812 | ||
| Sites Involved in Localized Disease | 813 | ||
| Bone | 814 | ||
| Lymph Node | 815 | ||
| Thymus | 816 | ||
| Thyroid | 816 | ||
| Lung | 816 | ||
| Sites Involved in Disseminated Disease | 819 | ||
| Skin | 819 | ||
| Liver | 821 | ||
| Bone Marrow | 821 | ||
| Central Nervous System | 821 | ||
| Gastrointestinal Tract | 822 | ||
| Spleen | 822 | ||
| Langerhans Cell Disease and Macrophage Activation | 823 | ||
| Malignant Langerhans Cell Disease and Langerhans Cell Sarcoma | 824 | ||
| References | 826.e1 | ||
| 53 Other Histiocytic and Dendritic Cell Neoplasms | 827 | ||
| Chapter Outline | 827 | ||
| Histiocytic Sarcoma | 827 | ||
| Definition | 827 | ||
| Epidemiology | 828 | ||
| Etiology | 828 | ||
| Clinical Features | 828 | ||
| Morphology | 828 | ||
| Ultrastructure | 829 | ||
| Immunophenotype | 829 | ||
| Genetics and Molecular Findings | 829 | ||
| Postulated Cell of Origin | 829 | ||
| Clinical Course | 829 | ||
| Differential Diagnosis | 829 | ||
| Dendritic Cell Neoplasms | 829 | ||
| Follicular Dendritic Cell Sarcoma | 829 | ||
| Definition | 829 | ||
| Epidemiology | 829 | ||
| Etiology | 829 | ||
| Clinical Features | 831 | ||
| Gross Description | 831 | ||
| Morphology | 831 | ||
| Grading | 832 | ||
| Ultrastructure | 832 | ||
| Immunophenotype | 832 | ||
| Genetics and Molecular Findings | 833 | ||
| Postulated Cell of Origin | 833 | ||
| Clinical Course | 833 | ||
| Differential Diagnosis | 833 | ||
| Interdigitating Dendritic Cell Sarcoma | 833 | ||
| Definition | 833 | ||
| Epidemiology | 833 | ||
| Etiology | 834 | ||
| Clinical Features | 834 | ||
| Morphology | 834 | ||
| Grading | 834 | ||
| Enzyme Cytochemistry | 834 | ||
| Ultrastructure | 834 | ||
| Immunophenotype | 834 | ||
| Genetics and Molecular Findings | 834 | ||
| Postulated Cell of Origin | 835 | ||
| Clinical Course | 835 | ||
| Differential Diagnosis | 835 | ||
| Other Dendritic Cell Tumors | 835 | ||
| Disseminated Juvenile Xanthogranuloma | 835 | ||
| Definition | 835 | ||
| Epidemiology | 835 | ||
| Etiology | 835 | ||
| Sites of Involvement | 835 | ||
| Clinical Features | 836 | ||
| Morphology | 836 | ||
| Ultrastructure | 836 | ||
| Immunophenotype | 836 | ||
| Genetics and Molecular Findings | 836 | ||
| Postulated Cell of Origin | 836 | ||
| Clinical Course | 836 | ||
| Differential Diagnosis | 836 | ||
| Conclusion | 836 | ||
| References | 836.e1 | ||
| VI Immunodeficiency Disorders | 837 | ||
| 54 The Pathology of Primary Immunodeficiencies | 839 | ||
| Chapter Outline | 839 | ||
| Classification | 840 | ||
| Epidemiology | 840 | ||
| Approach to Diagnosis | 840 | ||
| Laboratory Assessment of Immune Status | 840 | ||
| Lymphoproliferative Disorders in Primary Immunodeficiencies | 840 | ||
| Pathogenesis of Lymphoproliferative Disorders | 842 | ||
| Polyclonal Activation of Lymphoid Proliferation | 842 | ||
| Abnormal Regulation of Lymphoid Proliferation by the Dysfunctional Immune System | 842 | ||
| Genetic Abnormalities | 843 | ||
| PREDOMINANTLY ANTIBODY DEFICIENCIES | 843 | ||
| X-Linked Agammaglobulinemia | 843 | ||
| VII Site-Specific Issues in the Diagnosis of Lymphoma and Leukemia | 885 | ||
| 57 Bone Marrow Evaluation for Lymphoma | 887 | ||
| Chapter Outline | 887 | ||
| Distinction between Benign Lymphoid Infiltrates and Lymphoma | 888 | ||
| Benign Lymphoid Aggregates | 888 | ||
| Morphology | 888 | ||
| Immunohistochemistry | 890 | ||
| Flow Cytometric Immunophenotyping | 891 | ||
| Molecular Diagnostic Studies | 892 | ||
| Unusual Reactive Lymphoid Infiltrates | 893 | ||
| Systemic Polyclonal Immunoblastic Proliferations | 893 | ||
| Reactive Polymorphic Lymphohistiocytic Proliferations | 893 | ||
| Non-Hodgkin’s Lymphoma | 894 | ||
| Incidence of Bone Marrow Involvement | 894 | ||
| Histologic Patterns of Bone Marrow Involvement | 894 | ||
| Characteristic Features of B-Cell Lymphomas Involving Bone Marrow | 895 | ||
| Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia | 895 | ||
| Lymphoplasmacytic Lymphoma and Waldenström’s Macroglobulinemia | 897 | ||
| Splenic Marginal Zone Lymphoma | 898 | ||
| Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue | 899 | ||
| Nodal Marginal Zone Lymphoma | 899 | ||
| Follicular Lymphoma | 899 | ||
| Mantle Cell Lymphoma | 900 | ||
| Diffuse Large B-Cell Lymphoma | 902 | ||
| Intravascular Large B-Cell Lymphoma | 904 | ||
| Primary Effusion Lymphoma | 904 | ||
| Burkitt’s Lymphoma | 904 | ||
| B-Lymphoblastic Leukemia/Lymphoma | 904 | ||
| Characteristic Features of T-Cell Lymphomas Involving Bone Marrow | 905 | ||
| T-Lymphoblastic Leukemia/Lymphoma | 905 | ||
| Extranodal NK/T-Cell Lymphoma | 905 | ||
| Enteropathy-Type T-Cell Lymphoma | 906 | ||
| Hepatosplenic T-Cell Lymphoma | 906 | ||
| Subcutaneous Panniculitis-Like T-Cell Lymphoma | 906 | ||
| Mycosis Fungoides and Sézary Syndrome | 906 | ||
| Angioimmunoblastic T-Cell Lymphoma | 907 | ||
| Peripheral T-Cell Lymphoma, Not Otherwise Specified | 907 | ||
| Anaplastic Large Cell Lymphoma | 909 | ||
| Characteristics of Hodgkin’s Lymphoma Involving Bone Marrow | 909 | ||
| Evaluation of Bone Marrow Specimens from Patients with An Established Diagnosis of Lymphoma | 911 | ||
| Primary Diagnosis of Lymphoma in Bone Marrow | 912 | ||
| Nonlymphoid Malignancies that Mimic Lymphomas | 915 | ||
| Metastatic Tumors | 915 | ||
| Systemic Mastocytosis | 915 | ||
| References | 917.e1 | ||
| 58 Evaluation of the Bone Marrow After Therapy | 918 | ||
| Chapter Outline | 918 | ||
| General Approach | 918 | ||
| Acute Leukemia or Myelodysplasia | 920 | ||
| Acute Myeloid Leukemia and Myelodysplasia | 921 | ||
| Morphologic Features | 921 | ||
| Immunophenotyping | 922 | ||
| Cytogenetics and Molecular Studies | 923 | ||
| Acute Lymphoblastic Leukemia | 924 | ||
| Morphologic Features | 924 | ||
| Immunophenotyping | 924 | ||
| Cytogenetics and Molecular Studies | 925 | ||
| Distinguishing Hematogones | 925 | ||
| Chronic Myelogenous Leukemia | 927 | ||
| Morphologic Features | 927 | ||
| Immunophenotyping | 929 | ||
| Cytogenetics and Molecular Studies | 929 | ||
| Chronic Lymphoproliferative and Plasma Cell Disorders | 929 | ||
| Morphologic Features | 929 | ||
| Immunophenotyping | 930 | ||
| Cytogenetics and Molecular Studies | 931 | ||
| Other Bone Marrow Changes after Therapy | 933 | ||
| Necrosis | 933 | ||
| Fibrosis | 933 | ||
| Serous Atrophy | 934 | ||
| Solid Tumors | 934 | ||
| Growth Factor Changes | 935 | ||
| Late Effects of Therapy, Including Secondary Malignancies | 936 | ||
| References | 938.e1 | ||
| 59 Nonhematopoietic Neoplasms of the Bone Marrow | 939 | ||
| Chapter Outline | 939 | ||
| Metastatic Tumors in Adults | 941 | ||
| Metastatic Tumors in Children and Adolescents | 945 | ||
| Bone Tumors | 947 | ||
| Vascular and Miscellaneous Tumors | 948 | ||
| Benign Tumor-Like Lesions | 948 | ||
| Metabolic Bone Disease | 949 | ||
| Conclusion | 949 | ||
| References | 950.e1 | ||
| 60 Nonlymphoid Lesions of the Lymph Nodes | 951 | ||
| Chapter Outline | 951 | ||
| Metastatic Tumors in Lymph Nodes | 951 | ||
| Histologic Features of Metastatic Tumors | 951 | ||
| Characteristic Biologic Patterns of Metastasis | 953 | ||
| Role of Immunohistochemistry in the Diagnosis of Metastatic Tumors | 954 | ||
| Nonlymphoid Tumors with Prominent Reactive Lymphoid Components | 956 | ||
| Staging and Prognostic Factors in Lymph Node Metastasis | 957 | ||
| Sentinel Lymph Node Biopsy for Carcinoma | 957 | ||
| Lymph Node Dissection for Carcinoma | 957 | ||
| Sentinel Lymph Node Biopsy for Melanoma | 957 | ||
| Benign Lymph Node Inclusions | 958 | ||
| Epithelial and Mesothelial Inclusions in Lymph Nodes Adjacent to Solid Organs | 958 | ||
| Keratin-Positive Fibroblastic Reticular Cells | 958 | ||
| Nevus Cell Aggregates | 958 | ||
| Mesenchymal Proliferations in Lymph Nodes | 959 | ||
| Vascular Transformation of Lymph Node Sinuses and Lymphatic Proliferations | 959 | ||
| Mixed Smooth Muscle–Vascular Proliferations | 960 | ||
| Inflammatory Pseudotumor of Lymph Nodes | 961 | ||
| Kaposi’s Sarcoma | 961 | ||
| Vascular Tumors | 961 | ||
| Metastatic Sarcomas of Other Types | 962 | ||
| Bone Marrow Hematopoietic Elements and Tumors Involving Lymph Nodes | 962 | ||
| References | 964.e1 | ||
| 61 Spleen: | 965 | ||
| Chapter Outline | 965 | ||
| The Normal Spleen | 966 | ||
| Gross Examination | 967 | ||
| Diffuse Splenic Enlargement | 967 | ||
| White Pulp Involvement | 967 | ||
| Red Pulp Involvement | 967 | ||
| Focal Splenic Pathology | 967 | ||
| Splenic Rupture | 967 | ||
| Lymphoid Hyperplasia | 968 | ||
| Castleman’s Disease | 969 | ||
| Autoimmune Lymphoproliferative Syndrome | 969 | ||
| Hodgkin’s Lymphoma | 969 | ||
| Non-Hodgkin’s Lymphomas | 970 | ||
| Primary Splenic Lymphoma | 970 | ||
| Secondary Splenic Involvement by Lymphoma | 971 | ||
| Precursor Lymphoid Neoplasms | 971 | ||
| Mature B-Cell Lymphomas and Leukemias | 971 | ||
| Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma | 971 | ||
| B-Cell Prolymphocytic Leukemia | 972 | ||
| Lymphoplasmacytic Lymphoma | 972 | ||
| Mantle Cell Lymphoma | 973 | ||
| Follicular Lymphoma | 974 | ||
| Nodal and Extranodal Marginal Zone Lymphoma | 975 | ||
| Diffuse Large B-Cell Lymphoma | 975 | ||
| Burkitt’s Lymphoma | 975 | ||
| B-Cell Lymphoid Neoplasms Presenting with Prominent Splenomegaly | 975 | ||
| Splenic Marginal Zone Lymphoma | 975 | ||
| Hairy Cell Leukemia | 976 | ||
| Splenic Lymphoma/Leukemia, Unclassifiable | 976 | ||
| Splenic Diffuse Red Pulp Small B-Cell Lymphoma | 977 | ||
| Hairy Cell Leukemia Variant | 977 | ||
| Plasma Cell Neoplasms | 977 | ||
| Plasma Cell Myeloma | 977 | ||
| Primary Amyloidosis | 978 | ||
| Heavy-Chain Diseases | 978 | ||
| Mature T- and Nk-Cell Neoplasms | 978 | ||
| Angioimmunoblastic T-Cell Lymphoma | 979 | ||
| Anaplastic Large Cell Lymphoma | 980 | ||
| T-Cell Lymphoid Neoplasms Presenting with Prominent Splenomegaly | 980 | ||
| Hepatosplenic T-Cell Lymphoma | 980 | ||
| T-Cell Prolymphocytic Leukemia | 980 | ||
| T-Cell Large Granular Lymphocytic Leukemia | 980 | ||
| Myeloid Neoplasms | 981 | ||
| Acute Myeloid Leukemias | 981 | ||
| Chronic Myeloid Neoplasms | 981 | ||
| Chronic Myelogenous Leukemia | 982 | ||
| Polycythemia Vera | 982 | ||
| Primary Myelofibrosis | 982 | ||
| Essential Thrombocythemia | 983 | ||
| Other Chronic Myeloid Neoplasms | 984 | ||
| Systemic Mastocytosis | 984 | ||
| Proliferations of the Monocyte-Macrophage System | 984 | ||
| Hemophagocytic Syndromes | 984 | ||
| Histiocytic Sarcoma | 985 | ||
| “Malignant Histiocytosis” Associated with Mediastinal Germ Cell Tumor | 985 | ||
| Dendritic Cell Neoplasms | 985 | ||
| Follicular Dendritic Cell Sarcoma | 985 | ||
| Interdigitating Dendritic Cell Sarcoma | 986 | ||
| Langerhans Cell Histiocytosis | 986 | ||
| Tumor-Like and Nonhematopoietic Neoplastic Lesions | 986 | ||
| Splenic Cyst | 986 | ||
| Splenic Hamartoma | 986 | ||
| Inflammatory Pseudotumor | 986 | ||
| Sclerosing Angiomatoid Nodular Transformation | 987 | ||
| Peliosis | 987 | ||
| Splenic Vascular Tumor | 987 | ||
| Metastatic Tumors | 988 | ||
| Storage Diseases | 989 | ||
| References | 990.e1 | ||
| 62 Diagnosis of Lymphoma in Extranodal Sites Other Than Skin | 991 | ||
| Chapter Outline | 991 | ||
| Nervous System and Meninges | 991 | ||
| Central Nervous System | 991 | ||
| Epidemiology and Etiology | 991 | ||
| Clinical Features | 994 | ||
| Morphologic Features | 994 | ||
| Immunophenotype | 994 | ||
| Genetic Features | 995 | ||
| Postulated Normal Counterpart | 995 | ||
| Staging, Treatment, and Outcome | 995 | ||
| Differential Diagnosis | 995 | ||
| Eye | 995 | ||
| Clinical Features | 995 | ||
| Appendix Staining Techniques | 1021 | ||
| Setup | 1021 | ||
| Preparation of Buffy Coat Smears | 1021 | ||
| Preparation of Marrow Samples for Electron Microscopic Examination | 1021 | ||
| Staining Procedures | 1021 | ||
| Hematoxylin-Eosin Staining of Sections | 1021 | ||
| Reagents | 1021 | ||
| Fixatives | 1021 | ||
| Procedure | 1022 | ||
| Stains | 1022 | ||
| Wilder’s Reticulin Stain | 1022 | ||
| Reagents | 1022 | ||
| Fixatives | 1023 | ||
| Procedure | 1023 | ||
| Wright-Giemsa Stain for Smears | 1023 | ||
| Reagents | 1023 | ||
| Dip Procedure for Wright-Giemsa Stain with Slide Holders | 1023 | ||
| Iron Stains | 1024 | ||
| Dacie Method for Unstained Aspirate Smears | 1024 | ||
| Index | 1025 | ||
| A | 1025 | ||
| B | 1028 | ||
| C | 1031 | ||
| D | 1034 | ||
| E | 1035 | ||
| F | 1036 | ||
| G | 1037 | ||
| H | 1038 | ||
| I | 1041 | ||
| J | 1042 | ||
| K | 1042 | ||
| L | 1042 | ||
| M | 1045 | ||
| N | 1049 | ||
| O | 1050 | ||
| P | 1050 | ||
| Q | 1053 | ||
| R | 1053 | ||
| S | 1054 | ||
| T | 1056 | ||
| U | 1058 | ||
| V | 1058 | ||
| W | 1058 | ||
| X | 1058 | ||
| Z | 1058 |