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Global Hematology, An Issue of Hematology/Oncology Clinics of North America, E-Book

Global Hematology, An Issue of Hematology/Oncology Clinics of North America, E-Book

David J. Roberts | Sir David J. Weatherall

(2016)

Additional Information

Book Details

Abstract

This issue will focus on the global state of hematology and will include articles such as: The Global Burden of Anemia, Iron Deficiency Anemia: Problems in Diagnosis and Therapy, Progress Towards the Control and Management of the Thalassemias, Problems and Approaches for Blood Transfusion in the Developing Countries, and many more!

Table of Contents

Section Title Page Action Price
Front Cover Cover
Global Hematology\r i
Copyright\r ii
Contributors iii
CONSULTING EDITORS iii
EDITORS iii
AUTHORS iii
Contents vii
Preface: Global Hematology\r vii
Introduction: The Complexity and Challenge of Preventing, Treating, and Managing Blood Diseases in the Developing Countries\r vii
The Global Burden of Anemia\r vii
Iron Deficiency Anemia: Problems in Diagnosis and Prevention at the Population Level\r vii
The Present and Future Global Burden of the Inherited Disorders of Hemoglobin\r viii
Sickle Cell Disease in Sub-Saharan Africa\r viii
Progress Toward the Control and Management of the Thalassemias\r viii
Glucose-6-Phosphate Dehydrogenase Deficiency\r viii
Hematologic Changes Associated with Specific Infections in the Tropics\r ix
Global Approach to Hematologic Malignancies\r ix
Hematological Practice in India\r ix
Hematological Practice in Hong Kong and China\r ix
Hematology in Africa\r x
Problems and Approaches for Blood Transfusion in the Developing Countries\r x
Improving Laboratory and Clinical Hematology Services in Resource Limited Settings\r x
HEMATOLOGY/ONCOLOGY\rCLINICS OF NORTH AMERICA\r xi
FORTHCOMING ISSUES xi
June 2016 xi
August 2016 xi
October 2016 xi
RECENT ISSUES xi
February 2016 xi
December 2015 xi
October 2015 xi
Preface:Global Hematology\r xiii
Introduction: The Complexity and Challenge of Preventing, Treating, and Managing Blood Diseases in the Developing Countries 233
Key points 233
PREVALENCE AND MULTIPLE CAUSES OF ANEMIA IN THE DEVELOPING WORLD 234
HEMATOLOGIC CHANGES ASSOCIATED WITH SPECIFIC INFECTIONS IN THE TROPICS 236
Malaria 236
Visceral Leishmaniasis (Kala-azar) 237
Schistosomiasis 237
Trypanosomiasis 237
Hookworm 237
Complex Hematology of Childhood Human Immunodeficiency Virus Infection in the Developing Countries 237
TROPICAL EOSINOPHILIA 238
HEMATOLOGIC ASPECTS OF MALABSORPTION IN THE TROPICS 238
HEMOSTATIC FAILURE AS A MANIFESTATION OF SYSTEMIC DISEASE IN THE TROPICS 240
Dengue 240
Other Viral Hemorrhagic Fevers 241
GEOGRAPHIC VARIABILITY IN THE DISTRIBUTION AND EXPRESSION OF HEMATOLOGIC DISEASE 241
Lymphoma and Leukemia 241
Hereditary Blood Disorders 242
Aplastic Anemia 242
Hematologic Disorders Unique to Certain Countries 243
SUMMARY 243
REFERENCES 243
The Global Burden of Anemia 247
Key points 247
INTRODUCTION 247
METHODS 248
Estimation of Overall Anemia 248
Etiologic Attribution of Anemia Cases 249
Uncertainty and Aggregation 251
RESULTS 251
SUMMARY AND DISCUSSION 301
ACKNOWLEDGMENTS 304
REFERENCES 306
Iron Deficiency Anemia 309
Key points 309
EPIDEMIOLOGY OF ANEMIA 309
FRAMEWORK FOR ANEMIA CONTROL 312
OTHER MEASURES TO ADDRESS IRON DEFICIENCY ANEMIA 313
Delayed Cord Clamping 313
Malaria Control 313
Antihelminthic Therapy 314
Iron Enrichment of Cooked Foods 314
Benefits from Iron on Functional Health Outcomes 314
Risks of Iron Supplementation 315
Infection 315
Clinical trials data 315
Mechanistic evidence for increased risk from iron interventions on malaria 316
Experimental evidence for increased risk from iron interventions on diarrhea 317
Other risks of iron supplementation 318
MEASUREMENT OF IRON STATUS IN THE PUBLIC HEALTH CONTEXT 318
FUTURE DIRECTIONS 320
REFERENCES 321
The Present and Future Global Burden of the Inherited Disorders of Hemoglobin 327
Key points 327
INTRODUCTION 327
INHERITED DISORDERS OF HEMOGLOBIN 328
Structural Hemoglobin Variants 328
Hemoglobin S 328
Hemoglobin C 328
Hemoglobin E 329
Thalassemias 329
α-Thalassemia 329
β-Thalassemia 329
GLOBAL HEALTH BURDEN 330
Awareness and Recognition 330
World Health Organization 330
Global Burden of Diseases, Injuries, and Risk Factors Study 331
Thalassaemia International Federation 331
Global Sickle Cell Disease Network, Caribbean Association for Researchers in Sickle Cell Disease and Thalassemia, and Sickl ... 331
Factors Affecting the Health Burden of the Inherited Disorders of Hemoglobin 332
Malaria selection 332
Consanguinity 332
Population growth 332
Epidemiologic transition 333
Human migrations 333
Population stratification 334
Current Health Burden 334
Newborn estimates 334
Population estimates 334
Future Health Burden 336
Preventing affected births 336
Managing affected births 337
Carrier detection 337
SUMMARY 337
ACKNOWLEDGMENTS 338
REFERENCES 338
Sickle Cell Disease in Sub-Saharan Africa 343
Key points 343
INTRODUCTION 343
ORIGINS OF THE SICKLE MUTATION 344
SICKLE CELL DISEASE IN SUB-SAHARAN AFRICA 345
THE NATURAL HISTORY OF SICKLE CELL DISEASE IN AFRICA 346
MALARIA 346
BACTEREMIA 348
THE FUTURE OF SICKLE CELL DISEASE IN SUB-SAHARAN AFRICA 349
BOTTLENECKS IN THE CARE OF SICKLE CELL DISEASE IN SUB-SAHARAN AFRICA 349
FUTURE PRIORITIES FOR SICKLE CELL DISEASE IN AFRICA 351
SPECIFIC TREATMENTS FOR SICKLE CELL DISEASE IN SUB-SAHARAN AFRICA 351
SUMMARY 352
REFERENCES 353
Progress Toward the Control and Management of the Thalassemias 359
Key points 359
PROGRESS OF THALASSEMIA CONTROL IN THAILAND 359
Thalassemia in Thailand 359
PREVENTION AND CONTROL PROGRAM FOR THALASSEMIA IN THAILAND 361
THALASSEMIA IN OTHER REGIONS OF ASIA 362
GLOBAL ISSUES 369
SUMMARY 370
REFERENCES 370
Glucose-6-Phosphate Dehydrogenase Deficiency 373
Key points 373
INTRODUCTION 373
BIOCHEMISTRY OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE AND GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY 374
MOLECULAR-GENETIC BASIS OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY 376
EVOLUTION 379
EPIDEMIOLOGY AND MALARIA SELECTION 380
CLINICAL MANIFESTATIONS OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY 381
MANAGEMENT OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY 384
ANIMAL MODELS AND DRUG SCREENING 384
TESTING FOR GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY AND MALARIA CONTROL 386
CONCLUSION 387
REFERENCES 388
Hematologic Changes Associated with Specific Infections in the Tropics 395
Key points 395
MALARIA 396
The Life Cycle of Malaria Infection and Human Infection 396
SEVERE MALARIA 397
Malarial Anemia 398
Role of Hemolysis 398
Role of the Spleen 399
Massive Intravascular Hemolysis 400
Decreased Erythrocyte Deformability 400
Immune Complex–Mediated Hemolysis 400
Defective Marrow Response 401
Coagulation Abnormalities 403
MALARIA IN PREGNANCY 403
MALARIA IN SPLENECTOMIZED PATIENTS 404
HYPERREACTIVE MALARIAL SPLENOMEGALY SYNDROME 404
ASPECTS OF THE CLINICAL MANAGEMENT OF MALARIAL ANEMIA 405
VISCERAL LEISHMANIASIS (KALA-AZAR) 406
SCHISTOSOMIASIS 407
TRYPANOSOMIASIS 408
American Trypanosomiasis (Chagas Disease) 408
African Trypanosomiasis 408
HOOKWORM 409
BARTONELLOSIS 410
SUMMARY 411
REFERENCES 411
Global Approach to Hematologic Malignancies 417
Key points 417
GLOBAL APPROACH TO PEDIATRIC HEMATOLOGIC MALIGNANCIES 417
THE ROLE OF ESSENTIAL MEDICINES 418
BUILDING CAPACITY 419
Acute Lymphoblastic Leukemia 420
Incidence 420
Treatment and outcomes 421
Core resources required to treat acute lymphoblastic leukemia in low- and middle-income countries 423
Future directions 423
Changes in care delivery 423
Improved parent/child support systems 424
Acute Myelogenous Leukemia 424
Incidence 424
Treatment and outcomes 424
Core resources required to treat acute myelogenous leukemia in low- and middle-income countries 424
Chronic Myelogenous Leukemia 425
Future directions 425
Hodgkin Lymphoma 425
Incidence 425
Treatment and outcome 425
Non-Hodgkin Lymphoma 426
Incidence 426
Treatment and outcomes 426
Core resources required to treat B-cell lymphoma in low- and middle income countries 427
Future directions 427
REFERENCES 427
Hematological Practice in India 433
Key points 433
INTRODUCTION 433
DISORDERS OF RED CELLS 434
Nutritional Anemias 434
Thalassemias and Hemoglobinopathies 435
Other Anemias 435
Hereditary Hemochromatosis 435
DISORDERS OF HEMOSTASIS AND COAGULATION 436
Inherited Coagulation Disorders 436
Qualitative Disorders of Platelets 436
Management of Bleeding Disorders 436
Thrombotic Disorders 437
HEMATOLOGIC MALIGNANCIES 437
Myeloproliferative Disorders 437
Acute Leukemias 439
Multiple Myeloma 440
Lymphoproliferative Neoplasms 440
SUMMARY 441
REFERENCES 441
Hematological Practice in Hong Kong and China 445
Key points 445
INTRODUCTION 445
Health Care Systems in China and Hong Kong 446
Burden of Hematological Diseases 446
NONMALIGNANT HEMATOLOGICAL DISEASES: GENETIC DISORDERS 446
Thalassemias 446
Prenatal Diagnosis for Thalassemias 446
Preimplantation Genetic Diagnosis 447
Preimplantation Genetic Diagnosis of α-Thalassemia 447
Preimplantation Genetic Diagnosis of β-Thalassemia 447
Transfusion and Iron Chelation Therapy for Thalassemia Major 447
Hematopoietic Stem Cell Transplantation for Thalassemia Major 448
Hemophilia 448
Prenatal Diagnosis for Hemophilias 448
Treatment of Hemophilias 448
Glucose-6-Phosphate Dehydrogenase Deficiency 449
MALIGNANT HEMATOLOGICAL DISEASES 449
Different Patterns of Lymphomas 449
Epidemiology and Pathology of Natural Killer/T-cell Lymphomas 449
Advances in Natural Killer/T-cell Lymphomas 450
Treatment of Natural Killer/T-cell Lymphomas 450
Monitoring of Treatment Outcome 450
Prognostication of Natural Killer/T-cell Lymphomas 450
Other T-cell Lymphoid Malignancies 451
Acute Promyelocytic Leukemia: The Chinese Revolution 451
All-Trans Retinoic Acid in Induction Treatment of Newly Diagnosed Acute Promyelocytic Leukemia: The Shanghai Connection 451
Relapse of Acute Promyelocytic Leukemia After All-Trans Retinoic Acid Therapy 452
As2O3 in Relapsed Acute Promyelocytic Leukemia: The Harbin Connection 452
Role of As2O3 in Newly-Diagnosed Acute Promyelocytic Leukemia 452
Oral As2O3: The Hong Kong Connection 452
Oral-As2O3 Maintenance of Acute Promyelocytic Leukemia in CR1 (First Complete Remission) Decreases Relapses 452
DEVELOPING STRATEGIES 453
HAPLOIDENTICAL ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION: SCIENCE DRIVEN BY CLINICAL NEED 453
SUMMARY 453
REFERENCES 453
Hematology in Africa 457
Key points 457
INTRODUCTION 457
RED CELL DISORDERS 458
Anemia 458
Malaria 459
Pregnancy 460
Iron Deficiency Anemia 461
Hyperreactive Malarial Splenomegaly 461
Inherited Red Cell Disorders 461
Sickle Cell Disease 462
Nutritional Anemia 464
Iron Deficiency Anemia 464
Folate Deficiency 465
Vitamin B12 Deficiency 465
BONE MARROW DEPRESSION OR APLASIA 465
WHITE BLOOD CELL DISORDERS 466
Malignant White Cell Disorders 466
Chronic Myeloid Leukemia 467
Chronic Lymphocytic Leukemia 467
Adult T-Cell Leukemia and Lymphoma 467
PLATELET DISORDERS 468
Vascular Purpura 468
HEMOSTASIS AND THROMBOSIS 468
Coagulopathies 468
Thromboembolic Disease 469
Anticoagulation Therapy 469
Snake Bite 469
Low Prothrombin and Vitamin K–Dependent Coagulation Factors 469
Hemophilia 470
von Willebrand Disease 470
BLOOD TRANSFUSION 470
SUMMARY 471
REFERENCES 471
Problems and Approaches for Blood Transfusion in the Developing Countries 477
Key points 477
INTRODUCTION 477
SAFETY AND SUPPLY 478
BLOOD-BORNE INFECTIONS 478
Blood-Borne Viral Infections 479
Other Infections 479
TESTING BLOOD PRODUCTS 480
SUPPLY AND USAGE 480
Blood Donors 480
Use of Blood Products 481
Systems 483
Focus on Sub-Saharan Africa 483
IMPROVEMENTS 484
Putting the World Health Organization Objectives into Practice 484
Development of a National Transfusion Service in Nigeria 484
Improving the Blood Supply 485
Improving Screening for Blood-Transmitted Infections 486
Meeting the Financial Requirements of Transfusion Services 486
Improving the Clinical Use of Blood 487
The Ebola Pandemic in West Africa 488
Research Directions in Transfusion Medicine 488
SUMMARY 489
REFERENCES 490
Improving Laboratory and Clinical Hematology Services in Resource Limited Settings 497
Key points 497
BACKGROUND 497
IMPROVING CLINICAL SERVICES IN RESOURCE-POOR SETTINGS 498
Expansion of Medical Education 498
Medical school 498
Pediatrics 498
Hematology 498
Possible approaches to change 499
Improved Delivery of Care 499
Organization of clinical facilities 499
Re-organization of the structure of the health care team 499
Emphasis on clinical skills and ‘low tech’ approaches to care 500
Data management 501
Increased access to essential medicines 501
Changes in attitude 501
IMPROVING LABORATORY SERVICES IN RESOURCE-POOR SETTINGS 502
Implementing Quality Improvement 503
Step 1 of Quality Improvement Cycle: Benchmarking 503
Step 2 of Quality Improvement Cycle: Identify Gaps or Deficiencies in Current Provision 505
Step 3 of Quality Improvement Cycle: Consider the Whole System - the Sample Journey 505
Step 4 of the Quality Improvement Cycle - Developing a Plan for Service Improvement 510
Steps 5 and 6 of the Quality Improvement Cycle: Test, Monitor, Implement and Sustain Successful Changes 510
SUMMARY 510
REFERENCES 510
FURTHER READINGS 512
Index 513