Chapter Outline QUALITATIVE DISORDERS Platelet Membrane Glanzmann Thrombasthenia: Defective Platelet Integrin α IIb β 3 Other Integrins in Inherited Platelet Disorders Bernard-Soulier Syndrome: Defective Glycoprotein Ib/IX Complex Platelet-Type von Willebrand…
Chapter Outline BASIC CONCEPTS OF FIBRINOLYSIS COMPONENTS OF THE FIBRINOLYTIC SYSTEM Plasminogen Plasminogen Activators Inhibitors of Fibrinolysis Cellular Receptors in Fibrinolysis THE FIBRINOLYTIC ACTIONS OF PLASMIN Fibrinogen Fibrin Tissue Plasminogen…
Chapter Outline INHERITED BONE MARROW FAILURE SYNDROMES ASSOCIATED WITH PANCYTOPENIA Fanconi Anemia Dyskeratosis Congenita Shwachman-Diamond Syndrome Cartilage-Hair Hypoplasia Pearson Syndrome Familial Marrow Dysfunction INHERITED BONE MARROW FAILURE SYNDROMES ASSOCIATED WITH…
Chapter Outline ACQUIRED HEMORRHAGIC DISORDERS Drug-Induced Bleeding Disseminated Intravascular Coagulation Liver Disease Vitamin K Deficiency Massive Transfusion Coagulopathy Acquired Inhibitors of Coagulation Proteins Acquired Inhibitors to Prothrombin—Hemorrhagic Lupus Anticoagulant Syndrome…
Chapter Outline INTRODUCTION CLINICAL EVALUATION Clinical Presentation of Bleeding Relevant Clinical History Age of Onset Family Bleeding History Medications PHYSICAL EXAMINATION LABORATORY EVALUATION Sample Collection and Technique and Preanalytical Variables…
Chapter Outline HEREDITARY THROMBOTIC DISORDERS Antithrombin III Deficiency Protein C Deficiency Protein S Deficiency Resistance to Activated Protein C and the Factor V Leiden Mutation Prothrombin G20210A Mutation HYPERHOMOCYSTEINEMIA DYSFIBRINOGENEMIAS…
Chapter Outline DEFINITION AND CLASSIFICATION OF PHAGOCYTES PHAGOCYTE DISTRIBUTION AND STRUCTURE Regulation of Myelopoiesis Granulocytes Mononuclear Phagocytes FUNCTION OF PHAGOCYTES Humoral Mediators of the Inflammatory Response Adhesion and Migration into…
Chapter Outline HEMOGLOBIN STRUCTURE AND FUNCTION Hemoglobin Primary Structure Hemoglobin Secondary Structure Hemoglobin Tertiary Structure The Prosthetic Heme Group Hemoglobin Quaternary Structure Dioxygen Binding Redox Reactions of Hemoglobin Ineffective Erythropoiesis…
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…
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…
