Williams Hematology: The Red Cell and Its Diseases

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WilliamsHematology The Red Cell and Its Diseases
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Copyright
Contributors
Preface
Part I Structure and Physiology of the Red Cell
	STRUCTURE AND COMPOSITION OF THE ERYTHROCYTE*
		SUMMARY
		ERYTHRON
		REFERENCES
	ERYTHROPOIESIS AND RED CELL TURNOVER
		SUMMARY
		HISTORY
		PHYLOGENY OF RED CELL PRODUCTION
		ONTOGENY OF RED CELL PRODUCTION
		CELLULAR COMPONENTS OF ERYTHROPOIESIS AND ITS REGULATION
		RED CELL LIFE SPAN
		REMOVAL OF ERYTHROCYTE ORGANELLES
		MICRORNAS (miRNAS) IN ERYTHROPOIESIS
		MEASUREMENTS OF RED CELL MASS
		MEASUREMENTS OF RED CELL PRODUCTION
		MECHANISMS OF DESTRUCTION OF ABNORMAL RED BLOOD CELLS
		FATE OF HEMOGLOBIN IN INTRAVASCULAR DESTRUCTION
		REFERENCES
Part II Classification of Red Cell Diseases
	CLINICAL MANIFESTATIONS AND CLASSIFICATION OF ERYTHROCYTE DISORDERS
		SUMMARY
		ANEMIA
		ERYTHROCYTOSIS
		REFERENCES
PART III Diseases of Red Cell Production
	APLASTIC ANEMIA: ACQUIRED AND INHERITED
		SUMMARY
		INTRODUCTION ACQUIRED APLASTIC ANEMIA
		ETIOLOGY AND PATHOGENESIS
		HEREDITARY APLASTIC ANEMIA
		OTHER INHERITED APLASTIC ANEMIAS
		REFERENCES
	PURE RED CELL APLASIA
		SUMMARY
		CONSTITUTIONAL PURE RED CELL APLASIA (DIAMOND-BLACKFAN ANEMIA)
		INTRODUCTION
		TRANSIENT APLASTIC CRISIS AND TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD
		ACQUIRED PURE RED CELL APLASIA
		REFERENCES
	ANEMIA OF CHRONIC DISEASE
		SUMMARY
		DEFINITION AND HISTORY
		EPIDEMIOLOGY
		ETIOLOGY AND PATHOGENESIS
		CLINICAL FEATURES
		LABORATORY FEATURES
		HYPOFERREMIA AND DECREASED SERUM TRANSFERRIN
		DIFFERENTIAL DIAGNOSIS
		THERAPY, COURSE, AND PROGNOSIS
		REFERENCES
	ERYTHROPOIETIC EFFECTS OF ENDOCRINE DISORDERS
		SUMMARY
		THYROID DYSFUNCTION
		ADRENAL GLAND DISORDERS
		GONADAL HORMONES
		PITUITARY GLAND DISORDERS
		HYPERPARATHYROIDISM
		REFERENCES
	PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
		SUMMARY
		DEFINITION AND HISTORY
		EPIDEMIOLOGY
		ETIOLOGY AND PATHOGENESIS
		CLINICAL FEATURES
		LABORATORY FEATURES
		DIFFERENTIAL DIAGNOSIS
		THERAPY
		REFERENCES
	FOLATE, COBALAMIN, AND MEGALOBLASTIC ANEMIAS
		SUMMARY
		FOLATE
		COBALAMIN
		MEGALOBLASTIC ANEMIAS
		REFERENCES
	IRON METABOLISM
		SUMMARY
		INTRODUCTION
		DISTRIBUTION OF IRON IN THE AVERAGE PERSON
		DIETARY IRON
		IRON ABSORPTION
		REFERENCES
	IRON DEFICIENCY AND OVERLOAD
		SUMMARY
		IRON DEFICIENCY
		IRON STORAGE DISEASE
		REFERENCES
	ANEMIA RESULTING FROM OTHER NUTRITIONAL DEFICIENCIES
		SUMMARY
		VITAMIN-DEFICIENCY ANEMIAS
		INTRODUCTION
		TRACE METAL DEFICIENCY
		ANEMIA OF STARVATION
		ANEMIA OF PROTEIN DEFICIENCY (KWASHIORKOR)
		ALCOHOLISM
		REFERENCES
	ANEMIA ASSOCIATED WITH MARROW INFILTRATION
		SUMMARY
		ETIOLOGY AND PATHOGENESIS
		DEFINITION AND HISTORY
		CLINICAL FEATURES
		LABORATORY FEATURES
		DIFFERENTIAL DIAGNOSIS
		THERAPY, COURSE, AND PROGNOSIS
		REFERENCES
Part IV Anemias Resulting Principally from Inherited Disorders
	THE HEREDITARY DYSERYTHROPOIETIC ANEMIAS
		SUMMARY
		DEFINITION AND HISTORY
		EPIDEMIOLOGY
		CONGENITAL DYSERYTHROPOIETIC ANEMIA, TYPE I
		CONGENITAL DYSERYTHROPOIETIC ANEMIA, TYPE II
		GENETICS
		TREATMENT, COURSE, AND PROGNOSIS
		CONGENITAL DYSERYTHROPOIETIC ANEMIA, TYPE III
		OTHER CONGENITAL DYSERYTHROPOIETIC ANEMIAS
		DIFFERENTIAL DIAGNOSIS
		REFERENCES
	ERYTHROCYTE MEMBRANE DISORDERS
		SUMMARY
		INTRODUCTION
		OVERVIEW OF THE ERYTHROCYTE MEMBRANE
		COMPONENTS OF THE ERYTHROCYTE MEMBRANE
		MEMBRANE ORGANIZATION
		CELLULAR DEFORMABILITY AND MEMBRANE STABILITY
		MEMBRANE PERMEABILITY
		ERYTHROCYTE MEMBRANE DISORDERS
		REFERENCES
	ERYTHROCYTE ENZYME DISORDERS
		SUMMARY
		DEFINITION AND HISTORY
		EPIDEMIOLOGY
		ETIOLOGY AND PATHOGENESIS
		CLINICAL FEATURES
		LABORATORY FEATURES
		DIFFERENTIAL DIAGNOSIS
		THERAPY
		COURSE AND PROGNOSIS
		REFERENCES
	THALASSEMIA: A DISORDER OF GLOBIN SYNTHESIS
		SUMMARY
		DEFINITIONS AND HISTORY
		EPIDEMIOLOGY AND POPULATION GENETICS
		ETIOLOGY AND PATHOGENESIS
		MOLECULAR BASIS OF THE THALASSEMIAS
		PATHOPHYSIOLOGY
		CLINICAL FEATURES
		LABORATORY FEATURES AND DIFFERENTIAL DIAGNOSIS OF THE THALASSEMIA SYNDROMES
		THERAPY, COURSE, AND PROGNOSIS
		PROGNOSIS
		NOVEL THERAPEUTIC APPROACHES
		THALASSEMIA AS A GLOBAL HEALTH PROBLEM
		PREVENTION
		REFERENCES
	DISORDERS OF HEMOGLOBIN STRUCTURE: SICKLE CELL ANEMIA AND OTHER HEMOGLOBINOPATHIES
		SUMMARY
		THE STRUCTURE AND FUNCTION OF NORMAL HEMOGLOBIN
		NOMENCLATURE OF ABNORMAL HEMOGLOBINS
		SICKLE CELL DISEASE
		OTHER ABNORMAL HEMOGLOBINS
		REFERENCES
	METHEMOGLOBINEMIA AND OTHER DYSHEMOGLOBINEMIAS
		SUMMARY
		METHEMOGLOBINEMIA
		SULFHEMOGLOBIN
		LOW-OXYGEN AFFINITY HEMOGLOBINS: A CAUSE OF CYANOSIS
		OTHER DYSHEMOGLOBINS
		NITRIC OXIDE AND NITRIC OXIDE HEMOGLOBINS
		REFERENCES
	POLYCLONAL AND HEREDITARY SIDEROBLASTIC ANEMIAS
		SUMMARY
		EPIDEMIOLOGY
		ETIOLOGY AND PATHOGENESIS
		DEFINITION AND HISTORY
		CLINICAL AND LABORATORY FEATURES
		TREATMENT
		REFERENCES
	THE PORPHYRIAS
		SUMMARY
		DEFINITION AND HISTORY
		ETIOLOGY AND PATHOGENESIS
		ERYTHROPOIETIC PORPHYRIAS
		ACUTE PORPHYRIAS
		REFERENCES
Part V Anemia as a Result of Exogenous Factors
	FRAGMENTATION HEMOLYTIC ANEMIA
		SUMMARY
		PREECLAMPSIA OR ECLAMPSIA AND HELLP SYNDROME
		DISSEMINATED MALIGNANCY
		HEART VALVE HEMOLYSIS
		OTHER CAUSES OF NONIMMUNE HEMOLYSIS
		REFERENCES
	ERYTHROCYTE DISORDERS AS A RESULT OF TOXIC AGENTS
		SUMMARY
		ERYTHROCYTE TOXINS ACTING THROUGH WELL-DEFINED MECHANISMS
		ERYTHROCYTE TOXINS ACTING THROUGH INCOMPLETELY DEFINED MECHANISMS
		REFERENCES
	HEMOLYTIC ANEMIA RESULTING FROM INFECTIONS WITH MICROORGANISMS
		SUMMARY
		MALARIA
		BARTONELLOSIS (OROYA FEVER)
		BABESIOSIS
		CLOSTRIDIUM PERFRINGENS SEPTICEMIA
		OTHER INFECTIONS
		REFERENCES
	HYPERSPLENISM AND HYPOSPLENISM
		SUMMARY
		HYPERSPLENISM
		HYPOSPLENISM
		REFERENCES
	HEMOLYTIC ANEMIA RESULTING FROM IMMUNE INJURY
		SUMMARY
		DEFINITION AND HISTORY
		EPIDEMIOLOGY
		ETIOLOGY AND PATHOGENESIS
		CLINICAL FEATURES
		LABORATORY FEATURES
		DIFFERENTIAL DIAGNOSIS
		THERAPY
		COURSE AND PROGNOSIS
		REFERENCES
	ALLOIMMUNE HEMOLYTIC DISEASE OF THE FETUS AND NEWBORN
		SUMMARY
		EPIDEMIOLOGY
		CLINICAL FEATURES OF HEMOLYTIC DISEASE OF THE FETUS AND NEWBORN
		PATHOPHYSIOLOGY
		ANTENATAL MONITORING
		THERAPY
		OUTCOME
		PREVENTION
		CONCLUSIONS
		REFERENCES
Part VI Polyclonal Erythrocytosis
	PRIMARY AND SECONDARY ERYTHROCYTOSES (POLYCYTHEMIAS)
		SUMMARY
		DEFINITION AND HISTORY
		EPIDEMIOLOGY
		ETIOLOGY AND PATHOGENESIS
		CLINICAL FEATURES
		LABORATORY FEATURES
		SECONDARY ERYTHROCYTOSIS
		DIFFERENTIAL DIAGNOSIS
		ERYTHROCYTOSES OTHER THAN POLYCYTHEMIA VERA
		COURSE AND PROGNOSIS
		REFERENCES
Part VII Red Cell Transfusion
	ERYTHROCYTE ANTIGENS AND ANTIBODIES
		SUMMARY
		DEFINITIONS AND HISTORY
		BLOOD GROUP SYSTEMS
		ANTIGEN EXPRESSION
		BIOCHEMISTRY OF ERYTHROCYTE ANTIGENS
		EFFECT OF ENZYMES AND OTHER CHEMICALS ON ERYTHROCYTE ANTIGENS
		GENETICS OF ERYTHROCYTE ANTIGENS
		RED CELL ANTIGENS IN HEALTH AND DISEASE
		ASSOCIATIONS OF RED CELL ANTIGENS WITH DISEASE
		ERYTHROCYTE ANTIBODIES
		CLINICAL SIGNIFICANCE OF ERYTHROCYTE ANTIBODIES
		SEROLOGIC DETECTION OF ERYTHROCYTE ANTIGENS AND ANTIBODIES
		REFERENCES
	BLOOD PROCUREMENT AND RED CELL TRANSFUSION
		SUMMARY
		OVERVIEW OF THE BLOOD SUPPLY SYSTEM IN THE UNITED STATES
		INTERNATIONAL PRACTICES
		PROCUREMENT OF PLASMA DERIVATIVES
		RECRUITMENT OF BLOOD DONORS
		WHOLE-BLOOD DONOR SCREENING
		COLLECTION OF WHOLE BLOOD
		BLOOD COMPONENTS
		SPECIAL BLOOD DONATIONS
		COLLECTION AND PRODUCTION OF BLOOD COMPONENTS BY APHERESIS
		SELECTION OF APHERESIS DONORS
		REACTIONS IN APHERESIS DONORS
		LABORATORY TESTING OF DONATED BLOOD
		SAFETY OF THE BLOOD SUPPLY
		RED CELL TRANSFUSIONS
		HEMOGLOBINOPATHIES
		HEMATOPOIETIC STEM CELL TRANSPLANT
		SOLID-ORGAN TRANSPLANT
		ADVERSE EFFECTS OF TRANSFUSIONS
		POTENTIAL EFFECT OF AGE OF RED CELLS ON TRANSFUSION OUTCOME
		PATIENT BLOOD MANAGEMENT
		REFERENCES