HEMATOLOGY

Sickle Cell Disease

Apr 1, 2019 by in HEMATOLOGY Comments Off on Sickle Cell Disease

Chapter Outline PATHOPHYSIOLOGY Hemoglobin S Mutation Hemoglobin S Polymer Effects on the Erythrocyte Membrane External Membrane Interactions Hemolysis and Nitric Oxide Depletion Acute and Chronic Inflammation Genetic Modifiers DIAGNOSIS Fetus…

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Porphyrias

Apr 1, 2019 by in HEMATOLOGY Comments Off on Porphyrias

Chapter Outline FORMATION OF THE PYRROLE The δ-Aminolevulinate Synthases δ-Aminolevulinate Dehydratase ASSEMBLY OF THE TETRAPYRROLE Porphobilinogen Deaminase Uroporphyrinogen III Synthase MODIFICATION OF THE TETRAPYRROLE SIDE CHAINS Uroporphyrinogen Decarboxylase Coproporphyrinogen Oxidase…

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Glucose-6-Phosphate Dehydrogenase Deficiency

Apr 1, 2019 by in HEMATOLOGY Comments Off on Glucose-6-Phosphate Dehydrogenase Deficiency

Chapter Outline G6PD IN RED BLOOD CELL METABOLISM Structure and Biochemistry of G6PD Genetics of G6PD Features of G6PD in Red Blood Cells Molecular Basis of G6PD Deficiency Epidemiology of…

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Paroxysmal Nocturnal Hemoglobinuria

Apr 1, 2019 by in HEMATOLOGY Comments Off on Paroxysmal Nocturnal Hemoglobinuria

Chapter Outline HISTORY EPIDEMIOLOGY MOLECULAR PATHOGENESIS Paroxysmal Nocturnal Hemoglobinuria and Complement Decay Accelerating Factor Membrane Inhibitor of Reactive Lysis Inherited Mutations of CD55 and CD59 Congenital Abnormalities of Genes Involved…

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Pyruvate Kinase Deficiency and Disorders of Glycolysis

Apr 1, 2019 by in HEMATOLOGY Comments Off on Pyruvate Kinase Deficiency and Disorders of Glycolysis

Chapter Outline HEXOKINASE DEFICIENCY Clinical Manifestations Biochemistry Genetics Therapy GLUCOSE PHOSPHATE ISOMERASE DEFICIENCY Clinical Manifestations Biochemistry Genetics and Inheritance Therapy PHOSPHOFRUCTOKINASE DEFICIENCY Clinical Manifestations Biochemistry Genetics and Inheritance Therapy ALDOLASE…

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Autoimmune Hemolytic Anemia

Apr 1, 2019 by in HEMATOLOGY Comments Off on Autoimmune Hemolytic Anemia

Chapter Outline PRIMARY AUTOIMMUNE HEMOLYTIC ANEMIA Incidence Natural History Clinical Manifestations Routine Laboratory Evaluation Serologic Laboratory Evaluation Differential Diagnosis Characteristics of Erythrocyte Autoantibodies Therapy Pathogenesis of Autoantibody Formation SECONDARY AUTOIMMUNE…

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Neonatal Jaundice and Disorders of Bilirubin Metabolism

Apr 1, 2019 by in HEMATOLOGY Comments Off on Neonatal Jaundice and Disorders of Bilirubin Metabolism

Chapter Outline INTRODUCTION BILIRUBIN METABOLISM Bilirubin Production and Transport Hepatic Uptake of Bilirubin Bilirubin Conjugation Bilirubin Excretion Enterohepatic Circulation of Bilirubin DIAGNOSIS OF HYPERBILIRUBINEMIA Total Serum Bilirubin Measurements Transcutaneous Bilirubinometry…

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Megaloblastic Anemia

Apr 1, 2019 by in HEMATOLOGY Comments Off on Megaloblastic Anemia

Chapter Outline DEFINITIONS HISTORY FOLATE Chemistry of Folates Folate Sources, Requirements, and Stores Physiology of Folates Biochemistry of Folates COBALAMIN (VITAMIN B 12 ) Chemistry of Cobalamins Cobalamin Sources, Requirements,…

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Diagnostic Approach to the Anemic Patient

Apr 1, 2019 by in HEMATOLOGY Comments Off on Diagnostic Approach to the Anemic Patient

Chapter Outline INTRODUCTION DEFINITION OF ANEMIA CLASSIFICATION OF ANEMIAS EVALUATION OF THE ANEMIC PATIENT Initial Encounter Selection of Appropriate Laboratory Tests Automated Cell Counting The Blood Film Introduction In this…

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