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PART Ⅰ CLINICAL13

CHAPTER 1 THE HISTORICAL DEVELOPMENT OF THE CONCEPTS OF HEMOSTASIS13

Vascular Contraction13

The Coagulation of the Blood-Historical Highlights16

The Pre-classical Theory Era(1666-1904)16

The Classical Theory Era(1904-1934)17

The Prothrombin Era(1934-1943)18

The Platelets20

Thrombus Formation21

Platelet＇s Role in Coagulation22

Clot Retraction23

Vasoconstriction Action of Platelets24

CHAPTER 2 THE COAGULATION OF THE BLOOD AND ITS ROLE IN HEMOSTASIS33

The Conversion of Fibrinogen to Fibrin by Thrombin33

The Formation of Thrombin from Prothrombin35

The Conversion of Prothrombin to Thrombin in Plasma37

The Quantitative Determination of Prothrombin38

The Two-stage Method38

The One-stage Method40

The Labile Factor42

Prothrombinogen44

Why is the Prothrombin Time Constant in Fresh Normal Plasma？44

Why Does the Prothrombin Time Change During Storage？45

Why is the Prothrombin of the Blood of Newborn Infants Low When Measured by the Two-stage Method and Normal When Determined by the One-stage Test？47

Why is the Prothrombin Time of Serum from Hemophilic or Thrombocytopenic Blood Shorter Than That of Plasma？48

The Prothrombinogen-Prothrombin Ratio49

Stable Factor51

Calcium53

Thromboplastin55

Tissue Thromboplastin and Plasma Thromboplastin60

The Autocatalytic Thrombinogenic Mechanism61

The Control of the Thrombinogenic Cycle65

The Probable Sequence of Events in Hemostasis67

CHAPTER 3 THE HYPOPROTHROMBINEMIC STATES78

History78

On the Recognition of the Hypoprothrombinemic State80

Prothrombin and Vitamin K81

The Synthesis of Vitamin K84

Congenital Hypoprothrombinemia87

Congenital Hypoprothrombinemia Vera Type Ⅰ87

Congenital Hypoprothrombinemia Type Ⅱ88

Congenital Stable Factor Deficiency92

Congenital Labile Factor Deficiency93

Heredity94

Treatment97

Hypoprothrombinemia of the Newborn98

Hypoprothrombinemia in Obstructive Jaundice and Biliary Fistula101

Hypoprothrombinemia in Sprue and Related Conditions102

Prothrombin Activity in Diseases of the Liver103

Hypoprothrombinemia in Pernicious Anemia105

Hypoprothrombinemia in Tuberculosis105

Hypoprothrombinemia Induced by Antivitamin K Compounds106

Mechanism of the Action of Dicumarol109

Safety of Dicumarol in the Antepartum Period119

Hypoprothrombinemia Induced by Various Drugs120

CHAPTER 4 HEMOPHILIA AND HEMOPHILIA-LIKE DISEASES134

Hemophilia134

History134

The Coagulation Defect in Hemophilia136

Diagnosis139

The Prothrombin Consumption Time141

The Prothrombin Consumption Time with Added Thromboplastin Heated to 60℃142

The Hemolysate Test144

The Definitive Diagnosis of True Hemophilia(Hereditary Hypothromboplastinogenemia)146

The Clinical Picture147

Hemarthrosis150

Hemorrhage into the Central Nervous System151

Hematuria152

Heredity154

Social Implications159

Treatment160

Treatment of Acute Hemarthrosis168

Acquired Hypothromboplastinogenemia Due to a Thromboplastinogen Inhibitor(Antithromboplastinogenemia)170

Nature and Mode of Action170

Clinical Picture174

Diagnosis174

Treatment176

Theoretical Significance177

Hereditary Hypothromboplastinemic States(Other Than Hemophilia)178

Plasma Thromboplastin Component(PTC)Deficiency，Christmas Disease，Hemophilia B，and Hemophiloid State C178

Diagnosis180

Procedure 1180

Procedure 2181

Procedure 3182

Procedure 4182

Hemophilia-like States in the Female185

Detection of Hemophilic Carriers187

CHAPTER 5 THROMBOCYTOPENIC PURPURA197

Thrombocytopenic Purpura197

Early History197

Clinical Picture200

Course201

Pathology202

Bone Marrow202

Spleen202

Diagnosis203

Platelet Count203

Prothrombin Consumption Time204

Clot Retraction204

Bleeding Time204

Tourniquet Test204

Hemostatic Defect in Thrombocytopenic Purpura207

Clotting Factor in Platelets208

Clot Retraction209

Causes of Thrombocytopenia210

Excessive Destruction of Platelets212

Thrombocytopenic Purpura and Estrogens217

Congenital Thrombocytopenic Purpura218

Secondary Thrombocytopenic Purpura221

Purpura Due to Drugs221

Whole Body Irradiation222

Blood Dyscrasias223

Infection224

Treatment of Secondary Thrombocytopenic Purpura226

Thrombocytopenia in Blood Dyscrasia227

Treatment of Primary Thrombocytopenia227

Thrombotic Thrombocytopenic Purpura243

History243

Clinical Picture243

Pathology245

Diagnosis246

Treatment246

Thrombocythemia Hemorrhagica250

History250

Clinical Picture250

Pathology251

Diagnosis253

Treatment253

CHAPTER 6 SECONDARY NON-THROMBOCYTOPENIC PURPURAS256

Schonlein-Henoch Syndrome256

History256

Clinical Picture257

Onset257

Exanthem257

Joint Symptoms257

Abdominal Symptoms258

Renal Involvement258

Pathology258

Diagnosis259

Treatment260

Purpura Fulminans261

Clinical Picture262

Pathology262

Diagnosis263

Treatment263

Purpura Simplex264

Treatment265

Senile Purpura265

CHAPTER 7 PSEUDOHEMOPHILIAS A AND B；TELANGIECTASIA270

Pseudohemophilia A270

History270

Clinical Picture272

Course273

Heredity274

Diagnosis274

Basic Defect275

Treatment277

Social Significance277

Pseudohemophilia B278

History278

Clinical Picture279

Heredity279

Diagnosis280

Basic Defect280

Treatment282

Significance282

Telangiectasia285

History285

Clinical Picture286

Time of Onset286

Type of Bleeding286

Course288

Heredity288

Diagnosis289

Basic Defects290

Treatment291

Significance of the Disease292

CHAPTER 8 AFIBRINOGENEMIA AND HYPERHEPARINEMIA296

Hereditary Afibrinogenemia297

History297

Clinical Picture297

Diagnosis298

Pathology298

Heredity299

Treatment300

General Considerations300

Acquired Hypofibrinogenemia301

History301

Hypofibrinogenemia Associated with Pregnancy301

Premature Separation of the Placenta302

Prolonged Retention of Dead Fetus303

Amniotic Fluid Embolism303

Coagulation Defect in Obstetrical Bleeding304

Differential Diagnosis of Obstetrical Bleeding305

Treatment306

Miscellaneous Types of Acquired Hypofibrinogenemia307

Physiological Function of Fibrinogen308

Fibrinolysis309

Fibrinolysin as a Cause of Hemorrhage312

Hemolytic Transfusion Reactions313

Probable Mechanism of Hemolysis on Coagulation315

Hyperheparinemia326

History326

Heparin Co-Factor326

Physiological R61e in Coagulation328

Determination of Heparin in Blood329

Clinical Hyperheparinemia330

Treatment333

CHAPTER 9 VENOUS THROMBOSIS337

History337

The Mechanism of Thrombosis339

The Antithrombins and Anticoagulants and Their Relation to Thrombosis346

The Normal Serum Antithrombin，Albumin X346

Fibrin，the Physiological Antithrombin347

Heparin348

Dieumarol348

Thrombophlebitis349

Treatment350

PART Ⅱ LABORATORY METHODS355

Introduction355

General Comments and Suggestions356

Test 1-Clotting Time360

Test 2-Recalcified Plasma Clotting Time363

Test 3-Bleeding Time365

Test 4-Platelet Count367

Test S-Clot Retraction370

Test 6-Tourniquet Test373

Test 7-Thromboplastin：Preparation375

Test 8-Prothrombin Time379

Test 9-Prothrombin by Adsorption and Elution388

Test 10-Prothrombin Adsorbents391

Test 11-Total Prothrombin Time394

Test 12-Labile Factor396

Test 13-Vitamin K Assay400

Test 14-Assay of Dicumarol405

Test 15-Prothrombin Consumption Time408

Test 16-Thromboplastinogen Activity Time(TAT)412

Test 17-Preparation of Erythrocytin and Thromboplastinogen415

Test 18-Assay of Thromboplastinogen418

Test 19-Antithromboplastinogen421

Test 20-Thromboplastin Generation Test423

Test 21-Preparation of Thrombin427

Test 22-Assay of Heparin431

Test 23-Antithrombin Activity434

Test 24-Fibrinogen Determination436

Test 25-Calcium and Ion Exchange Resins440