1;Contents;5 2;Contributors;7 3;1: Basics of Coagulation;8 3.1;Formation of Fibrin;8 3.2;The Old Pathways;8 3.2.1;The Players;9 3.2.2;"Quaternary Complex";10 3.3;Initiation of Coagulation;10 3.3.1;Thrombin;11 3.3.2;Fibrin Formation;11 3.4;Propagation;12 3.5;Fibrinolysis;12 3.5.1;Fibrinolytic Proteins;12 3.5.2;Fibrinolysis: The Process;13 3.6;Platelet Production and Life Span;13 3.6.1;Thrombopoietin (TPO);13 3.7;Functions of Platelets;14 3.7.1;Platelet Adhesion;14 3.7.1.1;About von Willebrand Factor;14 3.7.1.2;Summary of Platelet Adhesion;14 3.7.2;Platelet Storage;14 3.7.3;Platelet Aggregation;14 3.7.4;Platelet Surface;15 3.8;Natural Anticoagulants;15 3.9;Suggested Reading;16 4;2: Tests of Hemostasis and Thrombosis;17 4.1;Bleeding Disorders;17 4.1.1;Bleeding History;17 4.1.2;Specific Assays for Bleeding Disorders (Tables 2.1, 2.2, and 2.3);17 4.1.2.1;Prothrombin Time (PT);17 4.1.2.2;Activated Partial Thromboplastin Time (aPTT);18 4.1.2.3;Specific Factor Assays;19 4.2;Platelet Function;20 4.2.1;Bleeding Time;20 4.2.2;Platelet Function Analysis;20 4.2.3;Flow Cytometry;20 4.2.4;Specific Platelet Studies;20 4.2.5;Test for DIC;21 4.2.6;Thromboelastography (TEG) (Table 2.8);22 4.3;Thrombotic Disorder;22 4.3.1;Tests for APLA;23 4.3.1.1;Specific Assays;23 4.3.2;Hypercoagulable States;24 4.4;Suggested Reading;24 5;3: Bleeding Disorders: A General Approach;25 5.1;Suggested Reading;27 6;4: Hemophilia;28 6.1;Introduction;28 6.2;Pathophysiology and Classification;28 6.3;Clinical Presentation and Symptoms;29 6.3.1;Hemarthrosis;29 6.3.2;Intramuscular Hematomas;29 6.3.3;Cerebral Hemorrhage;29 6.4;Diagnosis;29 6.5;Therapy;30 6.5.1;Hemophilia A (Factor VIII Deficiency);30 6.5.2;Hemophilia B (Factor IX Deficiency);31 6.6;Prophylactic Factor Use;32 6.6.1;Emicizumab;32 6.7;Guidelines for Specific Types of Bleeds (Table 4.3);33 6.8;Surgery in the Patient with Hemophilia;34 6.9;Inhibitors;34 6.10;Management of Factor Inhibitors;35 6.11;Future Therapy;36 6.12;Conclusions;36 6.13;Suggested Reading;36 7;5: Von Willebrand Disease;37 7.1;Introduction;37 7.2;Pathogenesis and Classification;37 7.3;Signs and Symptoms;38 7.4;Testing;39 7.5;Therapy;40 7.6;Therapy by vWD Subtype;41 7.7;Pregnancy;42 7.8;Suggested Reading;42 8;6: Other Inherited Bleeding Disorders;43 8.1;Platelet Defects;43 8.1.1;Introduction;43 8.1.2;Defective Platelet Function;43 8.1.3;Congenital Thrombocytopenia;44 8.2;Named Platelet Disorders;44 8.2.1;Platelet Function Disorders with Normal Platelet Numbers;44 8.2.2;Thrombocytopenia (Large Platelets);44 8.2.3;Thrombocytopenia (Normal-Sized Platelets);45 8.2.4;Thrombocytopenia (Small Platelets);45 8.3;Less Common Coagulation Disorder;45 8.4;Suggested Reading;47 9;7: Acquired Bleeding Disorders;48 9.1;Thrombocytopenia;48 9.2;Diagnostic Approach (Table 7.2);49 9.3;Etiologies of Thrombocytopenia (Table 7.3);49 9.3.1;Decreased Production;49 9.3.1.1;Sequestration;50 9.3.1.2;Increased Destruction: Immune;50 9.3.2;Dysfunctional Platelets;51 9.4;Acquired Coagulation Factor Deficiency;52 9.4.1;Vitamin K Deficiency;53 9.4.2;Antibiotics;53 9.4.3;Malnutrition;54 9.4.4;Rat Poison/"Superwarfarin";54 9.5;Specific Acquired Factor Deficiencies;54 9.6;Acquired Von Willebrand Disease;56 9.7;Suggested Reading;57 10;8: Disseminated Intravascular Coagulation;58 10.1;Pathogenesis;58 10.2;Etiology;59 10.3;Clinical Presentation (Table 8.3);60 10.4;Diagnosis;60 10.5;Suggested Reading;62 11;9: Liver and Renal Disease;63 11.1;Liver Disease;63 11.1.1;Pathogenesis of Defects;63 11.1.2;Evaluation and Treatment of Coagulation Defects in Liver Disease;64 11.2;Preparation for Surgery;65 11.2.1;Liver Transplantation;65 11.3;Uremia;66 11.3.1;Pathogenesis;66 11.3.2;Evaluation;66 11.3.3;Therapy (Table 9.4);66 11.4;Suggested Reading;67 12;10: Cardiac Bypass and Ventricular Assist Devices/ECMO;68 12.1;Introduction;68 12.2;Preoperative Coagulation Defects;68 12.3;Cardiopulmonary Bypass;69 12.4;Prevention and Therapy;69 12.4.1;Approach to the Bleeding Bypass Patient (Table 10.3);70 12