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CHAPTER 1 Origin and Development of Blood Cells21

Blood Formation in the Fetus21

Theories of Blood Formation22

Fetal Hemoglobin23

Fetal Myoglobin25

Bone Marrow at Birth,Infancy,and Childhood25

CHAPTER 2 Blood Changes During Growth-Postnatal Period,Infancy,and Childhood27

Blood Changes in the Newborn Infant27

Physiologic Anemia of the Newborn Infant28

Bone Marrow Changes28

Blood Volume28

Hemoglobin Concentration28

Erythrocyte Count29

Hematocrit Percentage(Volume of Packed Red Cells)29

Size of Red Cells(MCV)and Hemoglobin Concentration(MCHC)30

Reticulocytes30

Normoblasts30

Platelets30

White Blood Cells30

Blood of Premature Infants31

CHAPTER 3 Blood Dyscrasias in Relation to Maternal-Fetal Interaction34

Hereditary Basis of Blood Diseases-Genetic and Environmental Influences34

Blood Dyscrasias in Relation to Congenital Anomalies-General Principles34

Hematologic Aspects of Maternal and Fetal Interaction-Placental Physiology and Defects35

Fetal Hemorrhage Into the Maternal Circulation(Nonhemolytic Anemia of the New-born Infant)35

Bleeding From the Placental Surface37

Placental Transmission of Antibodies and Isoagglutinins37

Placental Transmission of the L．E．Factor38

Plasma Proteins in the Fetus and Newborn Infant39

Immunologic Relationships39

Transplacental Passage of Drugs Affecting Blood Elements in the Newborn Infant40

CHAPTER 4 Erythrocytes-General Considerations43

Properties of the Erythrocyte43

Iron Content and Oxygen Capacity44

Porphyrins and Blood Disorders45

Rouleaux Formation and Sedimentation45

Electrolyte Considerations45

Erythrocyte Production47

Normal Destruction of Erythrocytes52

CHAPTER 5 Erythrocytes-Morphologic Abnormalities59

General Considerations59

Abnormalities in Size59

Abnormalities in Shape60

Miscellaneous Changes62

CHAPTER 6 Blood Groups66

Blood Group Factors66

Definitions of Terms in Relation to Blood Groups66

ABO Blood Group System67

Rh-Hr Blood Group System69

MN,Ss,and P Blood Group Systems71

Rh Variants72

CHAPTER 7 Transfusions in Pediatric Practice75

Significant Factors in Transfusion Therapy75

Indications for Transfusions75

Blood Volume-plasma,Total Circulating Hemoglobin,and Erythrocyte Mass76

Hemoglobin and Hematocrit Levels in Acute and Chronic Anemias77

Choice and Dosage of Whole Blood,Packed Erythrocytes,and Plasma77

Transfusions in Premature Infants79

Limitations and Hazards of Transfusions79

Transfusion Therapy in Hemorrhagic Disorders83

Notes on Technique and Preservation of Blood83

CHAPTER 8 Jaundice-Differential Diagnosis in the Neonatal Period85

Jaundice in the Early Neonatal Period86

Physiologic Jaundice(Icterus Neonatorum)86

Hyperbilirubinemia of the Newborn Infant Unrelated to Isoimmunization87

Relation of Vitamin K to Hyperbilirubinemia,Kernicterus,and Hemolytic Anemia88

Hereditary Spherocytosis88

Hereditary Nonspherocytic Hemolytic Disease89

Elliptocytic(Ovalocytic)Hemolytic Anemia89

Heinz-Body Anemia in the Newborn Infant89

Acute Hemolytic Anemia Related to Naphthalene89

Infections in Newborn Infants90

Cytomegalic Inclusion Disease90

Congenital Toxoplasmosis90

Chronic Idiopathic Jaundice(Dubin-Johnson Type,Dubin-Sprinz Disease)90

Jaundice in the Later Neonatal Period91

Prolonged Obstructive Jaundice91

Obstructive Jaundice Complicating Erythroblastosis-Inspissated Bile Syndrome92

Inspissated Bile Syndrome of Unknown Etiology93

Atresia of the Bile Ducts94

Management of Prolonged Obstructive Jaundice94

Galactosemia95

Congenital Familial Nonhemolytic Jaundice With Kernicterus95

Jaundice and Carotenemia96

Jaundice Due to Pyloric Stenosis96

Jaundice and Hypothyroidism96

Hematomas96

Miscellaneous96

CHAPTER 9 Erythroblastosis Fetalis(Hemolytic Anemia of the Newborn Infant)-General Considerations100

Definition100

Pathogenesis100

Clinical Features101

Detection of Early Jaundice of the Newborn Infant101

Kernicterus102

Pathology103

Maternal Antibodies-Pienatal Testing104

Maternal Anti-Rh Titer104

Effect of Previous Transfusions on the Mother105

Immunization in the Rh-Positive Mother and Infant105

Heterozygons and Homozygous Status of the Husband105

Different Types of Antibodies105

Transmission of Antibodies106

Coombs Test(Antiglobulin Test)106

Tests With Trypsinized Cells107

Elution107

Frequency of Blood Group Factors Causing Erythroblastosis107

Prognostic Considerations and Family Patterns of Severity108

Laboratory Findings-Blood109

CHAPTER 10 Erythroblastosis Fetalis(Hemolytic Anemia of the Newborn Infant)-Treatment114

Objectives of Treatment114

Indications for Exchange Transfusions115

Induction of Labor119

Technique of Exchange Transfusions120

Miscellaneous Treatment124

Total Management of the Infant With Erythroblastosis125

Treatment of the Infant in Cardiac Failure127

Exchange Transfusion in Physiologic Hyperbilirubinemia of the Full-Term and Premature Infant127

Exchange Transfusions as a Treatment of Poisonings128

ABO Erythroblastosis128

Clinical Features129

Laboratory Findings-Blood129

Relation of ABO Compatibility and Rh Immunization131

Treatment132

Differential Diagnosis133

CHAPTER 11 Anemias-General Considerations137

Classification137

Orientation138

Diagnosis138

Principles of Treatment155

Allergic Implications of Blood Disorders155

CHAPTER 12 Iron-Deficiency Anemia159

Etiology159

Relation of Physiologic Anemia of the Newborn Infant to Iron-Deficiency Anemia161

Clinical Features162

Laboratory Data163

Diagnosis164

Treatment165

Anemia of the Premature Infant169

Management170

Iron Transport-Serum Iron and Iron-Binding Capacity171

Iron-Binding Capacity of Plasma in Various Clinical Conditions172

Hemosiderosis and Hemochromatosis174

Diagnostic Procedures175

Treatment175

Transient Dysproteinemia(Copper Deficiency in Infants)175

Acute Iron Intoxication176

Iron-Deficiency Anemia in Patients With Cyanotic Congenital Heart Disease177

CHAPTER 13 Megaloblastic Anemia and Related Anemias181

Megaloblastic Anemia of Infancy182

Etiology182

Clinical Features183

Laboratory Findings183

Treatment183

Prognosis184

Juvenile Pernicious Anemia184

Etiology184

Clinical Manifestations185

Laboratory Findings185

Diagnosis186

Course and Prognosis186

Treatment186

Nutritional Megaloblastic Anemia187

Miscellaneous Megaloblastic Anemias187

Megaloblastic Anemia With Hermolytic Anemias187

Megaloblastic Anemia With Hemochromatosis188

Megaloblastic Anemia With Fish Tapeworm188

Megaloblastic Anemia Caused by Anticonvulsant Therapy188

Malabsorption Syndromes-Sprue(Tropical),Idiopathic Steatorrhea(Nontropical Sprue),and Celiac Disease188

Normoblastic Macrocytic Anemias189

CHAPTER 14 Hypoplastic and Aplastic Anemias192

General Considerations192

Classification192

Hypoplastic Anemias193

Idiopathic Hypoplastic Anemia-Pure Red Cell Anemia193

Definition193

Pathogenesis194

Clinical Features194

Laboratory Findings195

Pathologic Findings195

Diagnosis195

Treatment196

Acquired Hypoplastic Anemias198

Anemia Due to Infections,Drugs,Chemicals,Toxins,and Autoimmune and Allergic States198

Aplastic Crisis198

Anemia Resulting from Suppressive Effect of Multiple Transfusions on Erythropoiesis198

Miscellaneous198

Aplastic Anemia(Bone Marrow Failure,Refractory Anemia)199

Definition199

Etiology199

Idiopathic Aplastic Anemia199

Congenital Aplastic Anemia With Multiple Congenital Anomalies(Fanconi Type)200

Congenital Hypoplastic Anemia Without Associated Anomalies201

Acquired(Secondary)Aplastic Anemias201

Anemia Due to Antimicrobial and Other Chemotherapeutic Agents201

Anemia Due to Industrial and Household Chemicals201

Anemia Due to Irradiation202

Miscellaneous Causes202

Features Common to Idiopathic and Acquired Types of Aplastic Anemia202

Pathology202

Clinical Features202

Laboratory Findings203

Differential Diagnosis203

Management204

Course and Prognosis206

CHAPTER 15 The Hemolytic Anemias210

Definition-General Considerations of Pathogenesis210

Classification210

Principal Features of Increased Hemolysis211

Evidence of Increased Marrow Activity214

Additional Tests for Detecting Abnormal Hemolysis215

Congenital Hemolytic Syndromes218

Hereditary Spherocytosis(Congenital Hemolytic Jaundice,Congenital Hemolytic Anemia,Spherocytic Anemia,Chronic Acholuric Jaundice,Chronic Familial Jaundice)219

Definition219

Inheritance and Race219

Etiology and Pathogenesis219

Clinical Features220

Growth221

Laboratory Data221

Hereditary Spherocytosis in the Newborn Period222

Diagnosis223

Treatment223

Sporadic Congenital Spherocytosis Associated With Congenital Hypoplastic Throm-bocyopenia and Malformations224

Hereditary Nonspherocytic Hemolytic Anemia(Atypical Familial Hemolytic Anamia,Congenital Nonspherocytie Anemia)224

Hereditary Elliptocytosis With Hemolytic Anemia225

Clinical and Blood Findings226

Treatment227

Hemolytic Anemia Due to Enzyme Deftciency Following Administration of Drugs and Other Agents227

Nonhereditary Hemolytic Anemia-Paroxysmal Nocturnal Hemoglobinuria(Marchiafava-Micheli Syndrome)228

Acquired Hemolytic Anemias230

Autoimmune Hemolytic Anemia(Chronic Idiopathic Autoimmune Hemolytic Disease,Chronic Acquired Hemolytic Anemia)231

Clinical Features231

Blood Findings231

Serologic Findings232

Pathogenesis232

Destruction of Sensitized Red Cells233

Diagnosis233

Treatment233

Prognosis234

Paroxysmal Cold Hemoglobinuria235

Dysproteinemias235

Acute Acquired Hemolytic Anemia(Lederer＇s Anemia)235

Idiopathic Paroxysmal Myoglobinuria236

March Hemoglobinuria236

CHAPTER 16 The Hereditary Hemoglobinopathies243

Methods for Determining the Hemoglobin Types243

Designation of Hemoglobin Types245

Electrophoretic Mobility of the Individual Hemoglobins245

Fetal Hemoglobin246

Hereditary Aspects247

Relation of Genetic Composition to Clinical-Hematologic Variations248

Target Cells249

Syndromes Associated With the Abnormal Hemoglobins250

Sickle Cell Disease250

Sickling Phenomenon250

Pathogenesis252

Sickle Cell Trait253

Incidence and Geographic Distribution253

Clinical and Laboratory Features253

Sickling and Malaria253

Sickle Cell(Drepanocytic)Anemia254

Pathology254

Clinical Features255

Skeletal Changes257

Blood258

Crises259

Diagnosis260

Treatment261

Prognosis262

Thalassamia(Cooley＇s Anemia,Mediterranean Anemia,Erythroblastic Anemia,Hereditary Leptocytosis)262

Historical262

Nomenclature262

Race and Incidence262

Genetic Transmission263

Effect of the Gene for Thalassemia Upon Other Hemoglobins263

Clinical Types263

Fetal Hemoglobin in Patients With Thalassemia265

Pathogenesis265

Pathology265

Clinical Features266

Growth and Maturation268

Skeletal Changes268

Blood Picture270

Hemosiderosis and Hemochromatosis274

Red Cell Survival274

Diagnosis274

Course and Prognosis276

Treatment277

Homozygous Hemoglobin C Disease279

Hemoglobin C Trait279

Hemoglobin D280

Hemoglobin E Disease280

Hemoglobin G281

Hemoglobin H281

Hemoglobin I282

Hemoglobin J282

Hemoglobin M283

Miscellaneous Abnormal Hemoglobins283

Sickle Cell Variants283

Sickle Cell-Thalassemia Disease(Microdrepanocytic Anemia)283

Essential Features284

Clinical Findings285

Sickle Cell-Hemoglobin C Disease285

Sickle Cell-Hereditary Spherocytosis286

Sickle Cell-Hemoglobin D Disease286

Thalassemia Variants287

Thalassemia-Hemoglobin C Disease287

Thalassemia-Hemoglobin E Disease288

Thalassemia-Lepore Hemoglobin289

CHAPTER 17 Polycythemia,Methemoglobinemia,Sulfhemoglobinemia,and Miscellaneous Anemias299

Polyeythemia299

Relative Polycythemia299

Primary Polycythemia(Polycythemia Vera,Erythremia,Vaquez-Osier Disease)300

Benign Familial Polycythemia300

Secondary Polycythemia(Erythrocytosis,Erythrocythemia,Compensatory Polycythemia300

Methemoglobinemia301

Pathogenesis301

Congenital(Familial)Methemoglobinemia302

Congenital Methemoglobinemia Associated With Hemoglobin M303

Drug-Induced Methemoglobinemia303

Features Common to Methemoglobinemia303

Diagnosis303

Treatment303

Methemoglobinemia in Young Infants304

Sulfhemoglobinemia304

Miscellaneous Anemias305

Anemia of Chronic Renal Insufficiency305

Pathogenesis305

Laboratory Findings305

Bone Marrow306

Diagnosis306

Treatment306

Anemia of Infection306

Pathogenesis307

Blood Findings and Other Laboratory Data307

Clinical Features308

Treatment308

Anemia of Acute Hemorrhage308

Etiology308

Blood Picture308

Clinical Features309

Treatment309

Chronic Hemorrhagic Anemia309

Etiology309

Clinical and Laboratory Features309

Treatment310

Vitamin Deficiencies and Anemia310

Anemia of Hypothyroidism311

Blood Changes in Lead Poisoning311

CHAPTER 18 Leukocytes-Cell Types317

Growth and Multiplication317

Chemotactic Factors318

Functions-Phagocytosis and Antibody Formation318

Erythrophagocytosis319

L．E．Phenomenon319

Life Span of Leukocytes322

Leukoagglutinins322

Types of White Cells323

Granulocytic or Myeloid Series323

Lymphocytes331

Miscellaneous334

Degenerative and Toxic Cytoplasmic Changes337

Cell Stains339

CHAPTER 19 Leukopenia and Leukopenic Syndromes345

Pathogenesis345

Causes346

Treatment351

CHAPTER 20 Leukocytosis,Leukemoid Reactions,and Lymphocytosis354

Leukocytosis354

Leukemoid Reactions355

Lymphocytosis356

Acute Infectious Lymphocytosis357

Definition357

Age357

Etiology357

Epidemiology357

Pathology357

Clinical Features357

Incubation Period358

Laboratory Findings358

Differential Diagnosis361

Treatment and Prognosis363

Chronic Nonspecific Infectious Lymphocytosis(Low-Grade Fever Syndrome)363

Clinical Picture363

Blood363

Differential Diagnosis364

Treatment and Prognosis365

CHAPTER 21 Infectious Mononucleosis367

Definition367

Historical367

Pathology367

Clinical Features368

Laboratory Findings371

Differential Diagnosis375

Prognosis375

Treatment375

Recurrences376

CHAPTER 22 Leukemia-General Aspects and Clinical Features379

Classification of Leukemia in Childhood379

Incidence,Age Distribution,Sex,and Frequency of Types383

Etiology384

Preleukemic Stage of Leukemia387

Leukemia in the Newborn Period(Congenital Leukemia)387

Spontaneous Remissions388

Clinical Features389

Differential Diagnosis396

CHAPTER 23 Leukemia-Treatment404

Treatment404

Remissions-Criteria413

Detailed Program of Treatment413

Simplified Program of Treatment in Stem Cell(Lymphoblastic)Leukemia418

Bone Marrow Transplantation418

Laboratory Determinations419

Treatment of Nervous System Involvement419

Results of Treatment-Prognosis for Survival419

CHAPTER 24 Leukemia-Allied Disorders422

Infrequent Types of Leukemia422

Bone Marrow Replacement and Leukoerythroblastosis423

Myelofibrosis423

Osteopetrosis(Marble-Bone Disease,Albers-Schonberg Disease)424

Extramedullary Megakaryocytosis and Acute Megakaryocytic Leukemia424

Thrombocythemia425

Erythremic Myelosis(Di Guglielmo＇s Disease)425

Chloroma and Chloroleukemia426

Neoplasms of Lymphoid Tissue(Malignant Lymphomas)426

CHAPTER 25 Disorders of the Spleen and the Reticuloendothelial System436

Role of the Spleen in Blood Disorders436

Structure of the Spleen436

Normal Functions of the Spleen436

Splenic Aspiration438

Adrenalin Test in Diagnosis of Hypersplenic Syndromes438

Indications for Splenectomy438

Disorders of the Spleen439

Splenomegaly439

Hypersplenism440

Congenital Absence of the Spleen441

Primary Splenic Neutropenia441

Felty＇s Syndrome441

Primary Splenic Panhematopenia441

Chronic Congestive Splenomegaly(Banti＇s Syndrome,Portal Hypertension,Splenic Anemia)442

Etiology and Pathogenesis442

Collateral Circulation443

Pathology443

Clinical Features443

Laboratory Data444

Diagnosis444

Course and Prognosis444

Treatment444

Diseases of the Reticuloendothelial System445

Gaucher＇s Disease446

Pathology and Pathogenesis446

Clinical Features446

Blood448

Heredity448

Course and Treatment448

Niemann-Pick Disease448

Clinical Features448

Pathology and Pathogenesis449

Blood449

Heredity450

Treatment450

Letterer-Siwe Disease,Hand-Schüller-Christian Disease,and Eosinophilic Granuloma450

Letterer-Siwe Disease(Nonlipid Reticuloendotheliosis)450

Pathology450

Diagnosis451

Blood451

Treatment and Course452

Hand-Schüller-Christian Disease452

Clinical Features452

Pathology452

Diagnosis453

Course453

Treatment453

Eosinophilic Granuloma453

CHAPTER 26 Blood Coagulation458

Normal Hemostatic Mechanisms458

Disorders Due to a Deficiency of Factors Required for Thromboplastin Formation(Phase 1 of Coagulation)464

General Consideration of the Hemophilias464

Classic Hemophilia(Hemophilia A,AHG Defciency)464

Hereditary Aspects464

Clinical Aspects465

Hemarthrosis465

Management466

Treatment of Bleeding466

Treatment of Hemarthroses469

Mild Hemophilia470

Diagnosis470

Roentgenographic Findings470

Prognosis471

Vascular Hemophilia(yon Willebrand＇s Disease,Pseudohemophilia B)472

Plasma Thromboplastic Component Deficiency(Hemophilia B,Christmas Disease)473

Clinical and Laboratory Features473

Treatment473

Plasma Thromboplastin Antecedent Defciency(Hemophilia C)474

Clinical and Laboratory Features474

Treatment474

Disorders Due to a Deficiency of Factors Required for the Conversion of Prothrombin to Thrombin(Phase 2 of Coagulation)474

General Considerations474

Hypoprothrombinemia(Vitamin K and Prothrombin Deficiency)475

Congenital Deficiencies476

Idiopathic(Congenital)Hypoprothrombinemia476

Labile Factor Deficiency(Factor Ⅴ Deficiency,Parahemophilia,Owren＇s Disease)476

Stable Factor Deficiency(Factor Ⅶ Deficiency,Proconvertin Deficiency,Congenital Hypoproconvertinemia)477

Hemorrhagic Disease of the Newborn Infant(Hypoprothrombinemia in the Newborn Infant)477

Etiology-Prothrombin and Stable Factor Deficiency477

Clinical and Laboratory Features478

Differential Diagnosis478

Treatment479

Stuart-Prower Factor Deficiency480

Multiple Defects480

Capillary and Single Coagulation Factor Deficiency480

Multiple Factor Deficiencies481

Disorders Due to a Deficiency of Fibrinogen(Phase 3 of Coagulation)481

Congenital Afibrinogenemia481

Congenital Hypofibrinogenemia481

Acquired Fibrinogen Deficiency481

Hypofibrinogenemia in Patients With Congenital Heart Disease481

Fibrinolysis(Fibrinolytic Purpura)482

Laboratory Findings in Fibrinogen Deficiencies482

Treatment of Fibrinogen Deficiencies483

Circulating Anticoagulants483

Summary of Replacement Therapy of the Coagulation Disorders485

Epistaxis486

Procedure for Screening Potential Bleeders486

Laboratory Investigation of Coagulation Disorders487

General Considerations487