1 Table of Contents

Sectian A: Anemias 1 Iron Deficiency Anemia 4 Thalassemia Minor 6 Anemia Associated with Hypotransferrinemia 8 P-Thdassemia Major 10 Anemia Associated with Lead Poisoning 12 Thalassemia Intermedia 15 Anemia Associated with Copper Deficiency 17 Hemoglobin E Disease 19 Congenital Sideroblastic Anemia 22 Macmcytic Anemia Associated with Folate Deficiency 25 Macrocytic Anemia Associated with B,, Deficiency 28 Pernicious Anemia 31 Methemoglobinemia 34 Hemolytic Anemia Associated with G6PD Deficiency 36 Anemia Associated with Pyruvate Kinase Deficiency 39 Hemolytic Disease of the Newborn

' 42 Anemia Associated with Fetomaternd Hemorrhage ' 45 Autoimmune Hemolytic Anemia, Warm-Reactive 48 Autoimmune Hemolytic Anemia, Cold-Reactive

' 50 Donath-Landsteiner Hemolytic Anemia (Paroxysmal Cold Hernoglobinuria) 53 HemogIobin SC Disease

6 55 Hereditary Spherocytosis 57 Sickle Cell Anemia

1 66 Hereditary Elliptocytosis ' 62 Sickle-a-Thalassemia 1 65 Hereditary Pyropo~kilocytosis

68 Anemia Associated with Liver Disease 70 Sickle Cell Anemia with Leg Ulcer 72 Anemia of Chronic Rend Insufficiency 74 Acquired Acanthocytosis 76 Aplastic Anemia 79 Diamond-Blackfan Anemia 82 Congenital Dyserythropoetic Anemia) Type I1 84 Fanconi Anemia and Myelodysplastic Syndrome 87 Pure Red Cell Aplasia Associated with Parvovirus Infection 89 Hemochmmatosis, WE-Associated 92 Hemochromatosis) Non-HFE Associated

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Section B: Acute Leukemias 95 AcuteMyeloid Leukemia with t(8;21) 98 Acute Myeloid Leukemia with Mutated NPMl 101 Acute Promyelocytic Leukemia with t(1S; 17) (q22;q12) 104 Acute Myelomonocytic Leukemia 107 Acute Myeloid Leukemia with Myelodysplasia-Related Changes 11 0 Acute Monoblastic Leukemia 11 3 Acute Myeloid Leukemia, with inv(3) 11 6 Acute Megakaryoblastic Leukemia 1 19 Therapy-Related Acute Myeloid Leukemia 122 Acute Myeloid Leukemia, Acute Panmyelosis with Myelofibrosis Subtype 125 Revised Blastic Plasmacytoid Dendritic Cell Neoplasm 128 MyeloidLymphoid Neoplasms with Eosinophilia and PDGFRA Rearrangement 130 Acute Ph- Lymphoblastic Leukemia 133 B-Lymphoblastic Leukemia, Pediatric 136 T-Lymphoblastic Leukemia Pediatric 139 T-Lymphoblastic Leukemia 141 Acute Ph+ Lymphoblastic Leukemia 144 Acute Leukemia ofAmbiguous Lineage 146 T-Lymphoblastic Crisis of Chronic Myelogenous Leukemia

Section C: Chronic Myeloproliferative Neoplasms and Myelodysplastic Syndromes 149 Chronic Myelogenous Leukemia 152 Atypical Chronic Myelogenous Leukemia, BCR-ABLI Negative 155 Chronic Neutrophilic Leukemia 158 Polycythemia Vera 160 Essential Thrombocythemia 162 Primary Myelofibrosis 165 Systemic Mastocytosis with Associated Clonal Hematologic Non-Mast Cell-Lineage Disease 168 Congenital Polycythemia 170 Myeloproliferative Neoplasm, Unclassifiable 173 Refractory Anemia with Ring Sideroblasts 176 Refractory Anemia with Ring Sideroblasts Associated with Marked Thrombocytosis 178 Refractory Cytopenia with Multilineage Dysplasia 181 Refractory Anemia with Excess Blasts-2 184 Myelodysplastic Syndrome with Isolated del(5q) 187 Myelodysplastic Syndrome, Unclassifiable 189 Chronic Myelomonocytic Leukemia

Section D: Chronic Lymphoproliferative Disorders 192 Chronic Lymphocytic Leukemia 195 Large B-Cell Lymphoma with Underlying Chronic Lymphocytic Leukemia (Richter Transformation) 197 B-Cell Prolymphocytic Leukemia 199 Hairy Cell Leukemia 202 Post-Transplant Lymphoproliferative Disorder 205 T-Cell Prolymphocytic Leukemia 208 T-Cell Large Granular Lymphocytic Leukemia 21 0 Adult T-Cell Lymphoma/Leukemia 21 3 Autoimmune Lymphoproliferative Syndrome

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Section E: Lymphomas and Their Mimics 21 6 Classical Hodgkin Lymphoma with Paraneoplastic Neuromyotonia 21 9 Nodular Lymphocyte-Predominant Hodgkin Lymphoma 222 Lymphocy te-Rich Classical H o d g h Lymphoma 225 Mixed Cellularity Classical Hodgkin Lymphoma 227 Follicular Lymphoma 230 Mantle Cell Lymphoma 233 Gastric MALT Lymphoma with Associated H Pylori Infection 236 Burkitt Lymphoma 239 Diffuse Large B-Cell Lymphoma 241 Double-Hit High-Grade B-Cell Lymphoma 244 T-Lymphoblastic Lymphoma 246 Extranodal N m - C e l l Lymphoma, Nasal Type 249 Angioimmunoblastic T-Cell Lymphoma 252 Hepatosplenic T-Cell Lymphoma 255 Primary Cutaneous T-Cell Lymphoma, y-6 Type 258 Nodal Marginal Zone Lymphoma 261 Peripheralization of Follicular Lymphoma 264 Mycosis Fungoides/S&zary Syndrome 266 Follicular Hyperplasia 269 Rosai-Dorfman Disease 271 Kikuchi-Fujimoto Disease (Histiocytic Necrotizing Lymphadenitis) 274 Progressive Transformation of Germinal Centers 277 Castleman Disease

Section F: Plasma Cell Disorders 280 Monoclonal Gammopathy of Undetermined Significance 282 Plasmacytoma 284 Multiple Myeloma 286 IgM-Secreting Myeloma 288 POEMS Syndrome (P~l~neuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein and Skin

Changes) 291 Primary Amyloidosis 294 Light Chain Deposition Disease 297 Multiple Myeloma and Acquired von Willebrand Disease

Section G: Platelet Disorders 300 Immune Thrombocytopenic Purpura (ITP) 303 Glanzmann Thrombasthenia 305 Gray Platelet Syndrome 307 Wiskott-Aldrich Syndrome 309 6-Storage Pool Deficiency (Hermansky-Pudlak Syndrome) 31 1 Inherited Platelet Secretion Defect/Signal Transduction Defect 31 3 Acquired Platelet Function Defect-Induced by Selective Serotonin-Reuptake Inhibitor

Section H: Hematologic Infectious Diseases 31 5 Babesiosis 31 8 Anaplasmosis 320 Malaria (Plasmodium vivax) 323 Relapsing Fever Secondary to Borrelia hermsii 325 Disseminated Histoplasma capsulatum

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Section I: Miscellaneous Hematologic Conditions

328 Persistent Polyclonal B-Lymphocytosis 331 Infectious Mononucleosis 334 Alloimmune Neonatal Neutropenia 337 Leukemoid Reaction Associated with Growth Factor Therapy 339 Chronic Benign Neutropenia 342 Acute Graft-vs-Host Disease 346 Chronic Graft-vs-Host Disease 348 Donor Lymphocyte Infusion Therapy 351 Transient Abnormal Myelopoiesis 354 Pelger-Huet Anomaly 357 May-Hegglin Anomaly 359 HIVrnerapy-Related Myelodysplasia 361 Hemophagocytic Lymphohistiocytosis 364 Alveolar Rhabdomyosarcoma of the Bone Marrow 367 Lobular Carcinoma of the Breast, Metastatic to the Bone Marrow 370 Paroxysmal Nocturnal Hemoglobinuria 373 Chbdiak-Higashi Syndrome 376 Niemann-Pick Disease 378 Mucopolysaccharidosis Type I (Hurler Syndrome) 380 Primary Hyperoxaluria Involving Bone Marrow

Section J: Bleeding Disorders 382 Factor VII Deficiency 384 Hemophilia A (Factor VIII Deficiency) -Newborn Diagnosis 386 Hemophilia A (Factor VIII Deficiency) Carrier 388 Hemophilia B actor IX Deficiency) 390 Hemophilia B (Factor IX Deficiency) Carrier 392 Factor V Inhibitor 394 Factor VIII Inhibitor 396 Acquired Factor X Deficiency 399 Factor XI Deficiency 401 Factor XI1 Deficiency 403 Factor XI11 Deficiency 405 von Willebrand Disease, Variant 2 Normandy 407 Acquired von Willebrand Disease

Section K: Thrombophilias 408 Factor V Leiden 41 0 Antiphospholipid Antibody Syndrome 412 Prothrombin Gene G20210AMutation 414 Hereditary Protein C Deficiency, Type I 41 6 Antithrombin Deficiency 419 Protein S Deficiency

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Section L: Miscellaneous Hemostasis Disorders 421 Thrombotic Thrombocytopenic Purpura 423 Heparin-Induced Thrombocytopenia with Thrombosis 425 Disseminated Intravascular Coagulation (DIC) 427 HELLP Syndrome (Hemolysis, Elevated Liver Enzymes, Low Platelets Syndrome) 429 'Thrombotic 7hombocytopenic Purpura in a Patient with Sickle Cell Crisis 431 Coagulopathy Associated with Cirrhosis 433 Plasminogen Deficiency, Type 1 435 Congenital Dysfibrinogenemia 437 Ischemic Stroke, Secondary to Atherosclerosis From Elevated Lp(a) Levels 439 CYP2C9 Genotyping for Management of Coumadin Therapy

Self-study Challenge Cases ---

Presentation Case number Discussion 441 Case 1 468 442 Case 2 469 443 Case 3 471 444 Case 4 473 445 Case 5 474 446 Case 6 476 447 Case 7 477 448 Case 8 479 449 Case 9 48 1 450 Case 10 482 45 1 Case 11 484 452 Case 12 486 453 Casszn-48- - 454 Case 14 488 455 Case 15 490 456 Case 16 492 457 Case 17 494 458 Case 18 496 459 Case 19 498 460 Case 20 500 461 Case 21 502 462 Case 22 503 463 Case 23 504 464 Case 24 506 465 Case 25 508 466 Case 26 510 467 Case 27 514

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