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Anemia, An Issue of Medical Clinics of North America, E-Book

Anemia, An Issue of Medical Clinics of North America, E-Book

Thomas G. DeLoughery

(2017)

Additional Information

Abstract

This issue of Medical Clinics, guest edited by Dr. Thomas G. DeLoughery, is devoted to anemia. Articles in this issue include: Anemia: Evaluation and Diagnostic Tests; Anemia of Chronic Disease; B12/Folate Deficiency; Iron Deficiency; Myelodysplasia; Autoimmune Hemolytic Anemia; Congenital Hemolytic Anemia; Sickle Cell Disease; Thrombotic Microangiopathy; Unusual Anemias; and Transfusion Therapy.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Anemia\r i
Copyright\r ii
CME Accreditation Page iii
PROGRAM OBJECTIVE iii
TARGET AUDIENCE iii
LEARNING OBJECTIVES iii
ACCREDITATION iii
DISCLOSURE OF CONFLICTS OF INTEREST iii
UNAPPROVED/OFF-LABEL USE DISCLOSURE iii
TO ENROLL iii
METHOD OF PARTICIPATION iii
CME INQUIRIES/SPECIAL NEEDS iv
MEDICAL CLINICS OF NORTH AMERICA\r v
FORTHCOMING ISSUES v
May 2017 v
July 2017 v
September 2017 v
RECENT ISSUES v
January 2017 v
November 2016 v
September 2016 v
Contributors vii
CONSULTING EDITOR vii
EDITOR vii
AUTHORS vii
Contents ix
Foreword: This Is Not an Anemic Issue\r ix
Preface: Anemia: Things Have Changed! ix
Anemia: Evaluation and Diagnostic Tests ix
Anemia of Inflammation: A Review\r ix
Megaloblastic Anemias: Nutritional and Other Causes ix
Iron Deficiency Anemia x
Myelodysplastic Syndromes: Updates and Nuances x
Autoimmune Hemolytic Anemia x
Congenital Hemolytic Anemia x
Sickle Cell Disease: A Brief Update xi
Syndromes of Thrombotic Microangiopathy xi
Unusual Anemias xi
Blood Transfusion Therapy xi
Foreword:\rThis Is Not an Anemic Issue xiii
Preface:\rAnemia: Things Have Changed! xv
Anemia 263
Key points 263
ANEMIA: DEFINITION 263
SYMPTOMS AND SIGNS OF ANEMIA 263
COMPENSATION FOR ANEMIA 264
CLASSIFICATION 264
DIAGNOSTIC TESTS 265
Complete Blood Count 266
Peripheral Blood Smear Assessment 266
Reticulocyte Count 274
RED CELL INDICES: OLD AND NEW 275
BONE MARROW EXAMINATION 276
CYTOGENETICS AND MOLECULAR TESTS 276
TESTING FOR SPECIFIC CAUSES OF ANEMIA 277
Renal Disease 277
Anemia of Inflammation 278
Endocrine Disease 279
Iron Deficiency 279
Other Nutritional Deficiencies 279
Vitamin B12 deficiency 279
Folate deficiency 279
Copper 280
Thalassemia 280
Sickle Cell Disease and Other Hemoglobinopathies 280
Hemolysis 280
Plasma Cell Myeloma 281
Myelodysplastic Syndrome 281
A Rational Approach to Anemia 284
REFERENCES 284
Anemia of Inflammation 285
Key points 285
EPIDEMIOLOGY 285
Anemia in Critical Illness 285
Anemia in Obesity 286
Anemia in Cancer 286
PATHOPHYSIOLOGY 286
Regulation of Hepcidin 287
Iron Sequestration 289
Changes in Erythrocyte Membrane 289
NEW EXPERIMENTAL MODELS 289
EXPERIMENTAL TREATMENTS 292
REFERENCES 293
Megaloblastic Anemias 297
Key points 297
MEGALOBLASTIC ANEMIAS: NUTRITIONAL AND OTHER CAUSES 297
Introduction 297
Pathogenesis of Megaloblastic Anemia 298
Clinical Picture 299
Laboratory Features 299
CONDITIONS THAT MAY CONFUSE OR OBSCURE THE PICTURE OF MEGALOBLASTIC ANEMIA 301
Coexistent Microcytic Anemia 301
Acute Leukemia and Myelodysplasia 301
Attenuated Megaloblastic Anemia 301
Acute Megaloblastic Anemia 301
CAUSES OF VITAMIN B12 DEFICIENCY 302
Impaired Absorption 302
Helicobacter pylori Infection 302
Gastrectomy Syndromes 302
Hyperchlorhydria 302
Diseases of the Small Intestine 303
Inherited Disorders of Vitamin B12 Absorption 303
Miscellaneous Causes 303
Inherited Disorders of B12 Metabolism 303
Homocystinuria 304
Methylmalonic Aciduria and Homocystinuria 304
CLINICAL FEATURES OF B12 DEFICIENCY 304
Neurologic Abnormalities 304
Concealed B12 Deficiency 304
MANAGEMENT AND PROGNOSIS 305
Injected Vitamin B12 305
High-dose Oral Treatment 305
Special Circumstances 305
CAUSES OF FOLATE DEFICIENCY 305
Cause and Pathogenesis 305
Dietary Deficiency 306
Impaired Absorption 306
Other Intestinal Defects 306
Increased Requirements 306
Increase in Cell Turnover 307
CLINICAL FEATURES OF FOLATE DEFICIENCY 307
LABORATORY FEATURES OF FOLATE DEFICIENCY 307
OTHER EFFECTS OF FOLATE DEFICIENCY 307
Neural Tube Closure 307
INBORN ERRORS OF FOLATE METABOLISM 307
Hereditary Folate Malabsorption 307
Dihydrofolate Reductase Deficiency 308
Methionine Synthase (MTR) Deficiency 308
Methylene Tetrahydrofolate Reductase Deficiency 308
MANAGEMENT AND PROGNOSIS 308
DRUG-INDUCED MEGALOBLASTIC ANEMIA 308
OTHER RARE CAUSES OF MEGALOBLASTIC ANEMIA 309
Congenital Dyserythropoietic Anemia 309
Refractory Megaloblastic Anemia 309
Acute Erythroid Leukemia 309
Thiamine-responsive Megaloblastic Anemia 309
REFERENCES 309
Iron Deficiency Anemia 319
Key points 319
INTRODUCTION 319
IRON METABOLISM 319
EPIDEMIOLOGY AND ETIOLOGIES 320
SYMPTOMS 322
DIAGNOSIS 323
TREATMENT 324
INTRAVENOUS IRON 326
DETERMINING THE ETIOLOGY OF IRON DEFICIENCY 327
SUMMARY 328
REFERENCES 328
Myelodysplastic Syndromes 333
Key points 333
INTRODUCTION 333
CLINICAL VIGNETTE 1: DIAGNOSTIC CONSIDERATIONS IN MYELODYSPLASTIC SYNDROME 334
ASSESSMENT OF CLINICAL VIGNETTE 1 334
CLINICAL VIGNETTE 2: EVALUATING PATIENTS WHO FAIL HYPOMETHYLATING AGENTS 337
ASSESSMENT OF CLINICAL VIGNETTE 2 337
CLINICAL VIGNETTE 3: RISK-ADAPTED TREATMENT AND ALLOGENEIC BLOOD AND MARROW TRANSPLANT 341
ASSESSMENT OF CLINICAL VIGNETTE 3 342
SUMMARY AND FUTURE CONSIDERATIONS 345
REFERENCES 345
Autoimmune Hemolytic Anemia 351
Key points 351
INTRODUCTION 351
CLINICAL PRESENTATION AND DIAGNOSIS 352
TREATMENT 355
Corticosteroids 355
Splenectomy 355
Immunosuppressive Drugs 356
Anti-CD20 Therapy (Rituximab) 356
SUMMARY 357
REFERENCES 357
Congenital Hemolytic Anemia 361
Key points 361
INTRODUCTION 361
CLINICAL PRESENTATION 362
Baseline 362
Hemolytic Event 362
Aplastic Event 363
Complications 363
LABORATORY FEATURES 363
DIFFERENTIAL DIAGNOSIS 364
MEMBRANOPATHIES 364
Hereditary Spherocytosis 364
Pathophysiology 364
Epidemiology 366
Diagnosis 366
Disease severity 366
Clinical presentation 367
Treatment 367
Hereditary Elliptocytosis 367
ENZYMOPATHIES 367
Glucose-6-Phosphate Dehydrogenase Deficiency 368
Pathophysiology 368
Epidemiology 368
Disease severity 368
Diagnosis 368
Clinical presentation 369
Treatment 369
Pyruvate Kinase Deficiency 369
Pathophysiology 369
Epidemiology 369
Diagnosis 370
Clinical presentation 370
Treatment 370
HEMOGLOBINOPATHIES 370
THALASSEMIA 370
Alpha-Thalassemia 371
Sickle Cell Disease 375
Key points 375
INTRODUCTION 375
EPIDEMIOLOGY 376
PATHOPHYSIOLOGY 376
Molecular Mechanisms 376
Cellular Mechanisms 377
DISEASE SEVERITY AND PROGNOSIS 378
MANAGEMENT 382
Infection Prevention 382
Red Blood Cell Transfusions 382
Iron Chelation 384
Hydroxyurea 385
Hematopoietic Stem Cell Transplantation 386
Treatments in Development 386
ACKNOWLEDGMENTS 387
REFERENCES 387
Syndromes of Thrombotic Microangiopathy 395
Key points 395
INTRODUCTION 395
Classical Thrombotic Thrombocytopenic Purpura 396
Clinical presentation 396
Neurologic 396
Hematologic 396
Renal 397
Gastrointestinal 397
Pulmonary 397
Cardiac 397
Pathogenesis 397
CONGENITAL THROMBOTIC THROMBOCYTOPENIC PURPURA 398
HEMOLYTIC UREMIC SYNDROME 399
Typical Hemolytic Uremic Syndrome 399
Atypical Hemolytic Uremic Syndrome 399
Complement Factor H Point Mutations 400
Autoantibodies to Factor H 400
Membrane Cofactor Protein 400
Complement Factor B and C3 Convertase 400
Factor I 400
METABOLISM-MEDIATED MICROANGIOPATHY 401
COAGULATION-MEDIATED MICROANGIOPATHY 401
DRUG-RELATED THROMBOTIC MICROANGIOPATHY 402
STEM CELL TRANSPLANT–RELATED THROMBOTIC MICROANGIOPATHY 402
PREGNANCY RELATED THROMBOTIC SYNDROMES 403
UPCOMING THERAPIES 404
Recombinant ADAMTS13 404
Caplacizumab 405
Bortezomib 405
SUMMARY 405
REFERENCES 405
Unusual Anemias 417
Key points 417
THALASSEMIA 417
COPPER DEFICIENCY 420
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA 420
APLASTIC ANEMIA 421
PURE RED CELL APLASIA 424
ACQUIRED HEMOLYTIC ANEMIAS 426
SUMMARY 427
REFERENCES 427
Blood Transfusion Therapy 431
Key points 431
INTRODUCTION 431
RISKS OF BLOOD TRANSFUSION 432
INDICATIONS FOR RED BLOOD CELL TRANSFUSION THERAPY 433
Pediatric Patients 433
Adult Patients 435
Clinical Practice Guidelines 438
Improving Blood Utilization 440
SUMMARY 442
REFERENCES 442
Index 449