Hemorrhagic DiseasesPDF电子书下载

PART Ⅰ CLINICAL 13

CHAPTER 1 THE HISTORICAL DEVELOPMENT OF THE CONCEPTS OF HEMOSTASIS 13

Vascular Contraction 13

The Coagulation of the Blood-Historical Highlights 16

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

The Classical Theory Era(1904-1934) 17

The Prothrombin Era(1934-1943) 18

The Platelets 20

Thrombus Formation 21

Platelet＇s Role in Coagulation 22

Clot Retraction 23

Vasoconstriction Action of Platelets 24

CHAPTER 2 THE COAGULATION OF THE BLOOD AND ITS ROLE IN HEMOSTASIS 33

The Conversion of Fibrinogen to Fibrin by Thrombin 33

The Formation of Thrombin from Prothrombin 35

The Conversion of Prothrombin to Thrombin in Plasma 37

The Quantitative Determination of Prothrombin 38

The Two-stage Method 38

The One-stage Method 40

The Labile Factor 42

Prothrombinogen 44

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 Ratio 49

Stable Factor 51

Calcium 53

Thromboplastin 55

Tissue Thromboplastin and Plasma Thromboplastin 60

The Autocatalytic Thrombinogenic Mechanism 61

The Control of the Thrombinogenic Cycle 65

The Probable Sequence of Events in Hemostasis 67

CHAPTER 3 THE HYPOPROTHROMBINEMIC STATES 78

History 78

On the Recognition of the Hypoprothrombinemic State 80

Prothrombin and Vitamin K 81

The Synthesis of Vitamin K 84

Congenital Hypoprothrombinemia 87

Congenital Hypoprothrombinemia Vera Type Ⅰ 87

Congenital Hypoprothrombinemia Type Ⅱ 88

Congenital Stable Factor Deficiency 92

Congenital Labile Factor Deficiency 93

Heredity 94

Treatment 97

Hypoprothrombinemia of the Newborn 98

Hypoprothrombinemia in Obstructive Jaundice and Biliary Fistula 101

Hypoprothrombinemia in Sprue and Related Conditions 102

Prothrombin Activity in Diseases of the Liver 103

Hypoprothrombinemia in Pernicious Anemia 105

Hypoprothrombinemia in Tuberculosis 105

Hypoprothrombinemia Induced by Antivitamin K Compounds 106

Mechanism of the Action of Dicumarol 109

Safety of Dicumarol in the Antepartum Period 119

Hypoprothrombinemia Induced by Various Drugs 120

CHAPTER 4 HEMOPHILIA AND HEMOPHILIA-LIKE DISEASES 134

Hemophilia 134

History 134

The Coagulation Defect in Hemophilia 136

Diagnosis 139

The Prothrombin Consumption Time 141

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

The Hemolysate Test 144

The Definitive Diagnosis of True Hemophilia(Hereditary Hypothromboplastinogenemia) 146

The Clinical Picture 147

Hemarthrosis 150

Hemorrhage into the Central Nervous System 151

Hematuria 152

Heredity 154

Social Implications 159

Treatment 160

Treatment of Acute Hemarthrosis 168

Acquired Hypothromboplastinogenemia Due to a Thromboplastinogen Inhibitor(Antithromboplastinogenemia) 170

Nature and Mode of Action 170

Clinical Picture 174

Diagnosis 174

Treatment 176

Theoretical Significance 177

Hereditary Hypothromboplastinemic States(Other Than Hemophilia) 178

Plasma Thromboplastin Component(PTC)Deficiency，Christmas Disease，Hemophilia B，and Hemophiloid State C 178

Diagnosis 180

Procedure 1 180

Procedure 2 181

Procedure 3 182

Procedure 4 182

Hemophilia-like States in the Female 185

Detection of Hemophilic Carriers 187

CHAPTER 5 THROMBOCYTOPENIC PURPURA 197

Thrombocytopenic Purpura 197

Early History 197

Clinical Picture 200

Course 201

Pathology 202

Bone Marrow 202

Spleen 202

Diagnosis 203

Platelet Count 203

Prothrombin Consumption Time 204

Clot Retraction 204

Bleeding Time 204

Tourniquet Test 204

Hemostatic Defect in Thrombocytopenic Purpura 207

Clotting Factor in Platelets 208

Clot Retraction 209

Causes of Thrombocytopenia 210

Excessive Destruction of Platelets 212

Thrombocytopenic Purpura and Estrogens 217

Congenital Thrombocytopenic Purpura 218

Secondary Thrombocytopenic Purpura 221

Purpura Due to Drugs 221

Whole Body Irradiation 222

Blood Dyscrasias 223

Infection 224

Treatment of Secondary Thrombocytopenic Purpura 226

Thrombocytopenia in Blood Dyscrasia 227

Treatment of Primary Thrombocytopenia 227

Thrombotic Thrombocytopenic Purpura 243

History 243

Clinical Picture 243

Pathology 245

Diagnosis 246

Treatment 246

Thrombocythemia Hemorrhagica 250

History 250

Clinical Picture 250

Pathology 251

Diagnosis 253

Treatment 253

CHAPTER 6 SECONDARY NON-THROMBOCYTOPENIC PURPURAS 256

Schonlein-Henoch Syndrome 256

History 256

Clinical Picture 257

Onset 257

Exanthem 257

Joint Symptoms 257

Abdominal Symptoms 258

Renal Involvement 258

Pathology 258

Diagnosis 259

Treatment 260

Purpura Fulminans 261

Clinical Picture 262

Pathology 262

Diagnosis 263

Treatment 263

Purpura Simplex 264

Treatment 265

Senile Purpura 265

CHAPTER 7 PSEUDOHEMOPHILIAS A AND B；TELANGIECTASIA 270

Pseudohemophilia A 270

History 270

Clinical Picture 272

Course 273

Heredity 274

Diagnosis 274

Basic Defect 275

Treatment 277

Social Significance 277

Pseudohemophilia B 278

History 278

Clinical Picture 279

Heredity 279

Diagnosis 280

Basic Defect 280

Treatment 282

Significance 282

Telangiectasia 285

History 285

Clinical Picture 286

Time of Onset 286

Type of Bleeding 286

Course 288

Heredity 288

Diagnosis 289

Basic Defects 290

Treatment 291

Significance of the Disease 292

CHAPTER 8 AFIBRINOGENEMIA AND HYPERHEPARINEMIA 296

Hereditary Afibrinogenemia 297

History 297

Clinical Picture 297

Diagnosis 298

Pathology 298

Heredity 299

Treatment 300

General Considerations 300

Acquired Hypofibrinogenemia 301

History 301

Hypofibrinogenemia Associated with Pregnancy 301

Premature Separation of the Placenta 302

Prolonged Retention of Dead Fetus 303

Amniotic Fluid Embolism 303

Coagulation Defect in Obstetrical Bleeding 304

Differential Diagnosis of Obstetrical Bleeding 305

Treatment 306

Miscellaneous Types of Acquired Hypofibrinogenemia 307

Physiological Function of Fibrinogen 308

Fibrinolysis 309

Fibrinolysin as a Cause of Hemorrhage 312

Hemolytic Transfusion Reactions 313

Probable Mechanism of Hemolysis on Coagulation 315

Hyperheparinemia 326

History 326

Heparin Co-Factor 326

Physiological R61e in Coagulation 328

Determination of Heparin in Blood 329

Clinical Hyperheparinemia 330

Treatment 333

CHAPTER 9 VENOUS THROMBOSIS 337

History 337

The Mechanism of Thrombosis 339

The Antithrombins and Anticoagulants and Their Relation to Thrombosis 346

The Normal Serum Antithrombin，Albumin X 346

Fibrin，the Physiological Antithrombin 347

Heparin 348

Dieumarol 348

Thrombophlebitis 349

Treatment 350

PART Ⅱ LABORATORY METHODS 355

Introduction 355

General Comments and Suggestions 356

Test 1-Clotting Time 360

Test 2-Recalcified Plasma Clotting Time 363

Test 3-Bleeding Time 365

Test 4-Platelet Count 367

Test S-Clot Retraction 370

Test 6-Tourniquet Test 373

Test 7-Thromboplastin：Preparation 375

Test 8-Prothrombin Time 379

Test 9-Prothrombin by Adsorption and Elution 388

Test 10-Prothrombin Adsorbents 391

Test 11-Total Prothrombin Time 394

Test 12-Labile Factor 396

Test 13-Vitamin K Assay 400

Test 14-Assay of Dicumarol 405

Test 15-Prothrombin Consumption Time 408

Test 16-Thromboplastinogen Activity Time(TAT) 412

Test 17-Preparation of Erythrocytin and Thromboplastinogen 415

Test 18-Assay of Thromboplastinogen 418

Test 19-Antithromboplastinogen 421

Test 20-Thromboplastin Generation Test 423

Test 21-Preparation of Thrombin 427

Test 22-Assay of Heparin 431

Test 23-Antithrombin Activity 434

Test 24-Fibrinogen Determination 436

Test 25-Calcium and Ion Exchange Resins 440