Blombäck's Essential Guide to Blood Coagulation

New edition of a concise, comprehensive, easy-to-read pocket book distilling the most clinically up-to-date information on thrombotic and bleeding disorders

In a concise manner, Blombäck’s Essential Guide to Blood Coagulation distills the most clinically up-to-date information on thrombotic and bleeding disorders, including the latest treatment strategies for key conditions and diseases. This book covers both the stable and the acute stages of hereditary and acquired bleeding and thrombotic disorders in adults, children, and geriatric patients. It also highlights the laboratory tests that can aid in diagnosing and identifying the cause of bleeding and/or thrombosis in a patient.

The book is organized into four main sections, each featuring numerous updates in this new edition. The first part introduces the reader to laboratory hemostasis and hemostasis physiology. The second part covers bleeding disorders, including a new chapter on bleeding disorders and aging. The third part discusses thromboembolic disorders, with a new chapter on extracorporeal membrane oxygenation (ECMO). The final section addresses special issues in hemostasis, with new chapters on hemostasis in renal disease, liver disorders, and cancer, as well as updated information on thrombotic thrombocytopenic purpura.

This edition of Blombäck’s Essential Guide to Blood Coagulation also includes updates on:

• Hemostasis in COVID-19 and vaccine-induced thrombotic thrombocytopenia (VITT), a rare condition observed during pandemic
• Critical bleeding in different clinical situations and hemostasis in obstetrics and gynecology
• Platelet disorders, and rare coagulation factor deficiencies, cerebral vein and splanchnic vein thrombosis
• The greater paradigm shift in the treatment of hereditary bleeding disorders since the last edition was published
• Laboratory issues in hemostasis, including nomenclature and sampling
• Specific issues of hemostasis in children

Compact enough to fit into a coat pocket, Blombäck’s Essential Guide to Blood Coagulation is a valuable guide for all those treating patients to expand their knowledge of hemostatic disorders. This text fills a void for the residents, fellow doctors, and practicing physicians who need rapid access to up-to-date information.

Contributors, xi

Preface, xiii

Abbreviations, xv

PART 1: GENERAL HEMOSTASIS

1 Overview of normal hemostasis, 3

Jovan P. Antovic, Maria Farm, and Charlotte Gran

2 Sampling instructions and preanalytical issues in hemostasis, 6

Maria Farm and Charlotte Gran

Points to note prior to blood sampling, 6

Sampling time and patient preparation, 6

Referrals for coagulation analyses, 7

Sampling, 7

Sample handling, 8

3 Laboratory investigation, 9

Jovan P. Antovic, Maria Farm, Charlotte Gran, Bianca Tesi, and Agneta Wikman

Nomenclature, 9

Screening analyses, 9

Special analyses, 16

Platelet assays, 24

Genetic testing, 26

Global hemostatic assays, 28

Useful assays in research studies, 31

PART 2: BLEEDING DISORDERS

4 Clinical and laboratory investigation of increased bleeding tendency, 37

Charlotte Gran and Maria Magnusson

Introduction, 37

Clinical investigation, 37

Laboratory investigations, 39

HEREDITARY BLEEDING DISORDERS

5 Hemophilia, 45

Margareta Holmström and Susanna Ranta

Introduction, 45

Bleeding symptoms in hemophilia, 45

Treatment, 45

Clinical follow-up of patients with bleeding disorders, 51

6 Von Willebrand disease, 52

Tony Frisk and Margareta Holmström

Introduction, 52

Classification, 52

Diagnosis, 52

Management, 53

7 Rare bleeding disorders including inherited platelet function defects, 58

Cecilia Karlström and Maria Magnusson

Inherited platelet function defects, 58

Very rare coagulation factor deficiencies, 60

Vascular bleeding disorders, 63

Bleeding disorder of unknown cause (BDUC), 64

8 Specific issues of hemostasis in children – focus on bleeding, 65

Maria Farm and Susanna Ranta

Developmental hemostasis, 65

Bleeding in children, 65

Coagulation factor deficiencies, 68

Platelet function defects and thrombocytopenia, 69

9 Hereditary bleeding disorders and aging, 71

Jovan P. Antovic, Roza Chaireti, and Joel Ohm

Cardiovascular disease (CVD), 71

Atrial fibrillation, 71

Atherosclerotic cardiovascular disease (ASCVD), 72

Cancer, 73

Chronic kidney disease, 73

10 Paradigm change – new treatment for hereditary bleeding disorders, 75

Maria Magnusson and Susanna Ranta

Coagulation factor concentrates, 75

Nonfactor replacement therapy, 75

Gene therapy, 76

ACQUIRED BLEEDING DISORDERS

11 Acquired inhibitors of coagulation factors, 81

Roza Chaireti, Margareta Holmström, and Cecilia Karlström

Acquired hemophilia, 81

Acquired von Willebrand disease, 84

CRITICAL BLEEDINGS

12 Critical bleeding in trauma and surgery, 89

Andreas Andersson, Carl Magnus Wahlgren, and Agneta Wikman

Background, 89

Pathophysiology, resuscitation, and surgical management of traumatic bleeding, 89

Management of acute bleeding in surgery, 91

Treatment protocols, 91

Pediatric considerations, 92

13 Gastrointestinal bleeding, 93

Maria Magnusson and Per Stål

Background, 93

Diagnosis, 93

Acute management, 94

Diagnostic-therapeutic procedures, 94

Secondary prophylaxis after GI bleeding, 95

Considerations regarding hemostasis in GI bleeding, 95

14 Intracranial bleeding, 96

Michael V. Mazya and Michael Nekludov

Intracerebral hemorrhage, 96

Subarachnoid hemorrhage, 98

Traumatic brain injury, 99

15 Obstetric massive bleeding, 101

Maria Sennström

Background, 101

Management, 101

Specific treatments, 101

Follow-up, 102

16 Bleedings associated with anticoagulant and antiplatelet treatment

(including reversal of antithrombotic treatment), 103

Margareta Holmström and Joel Ohm

Background, 103

Management, 103

PART 3: THROMBOEMBOLIC DISORDERS

VENOUS THROMBOSIS

17 Venous thromboembolism, 111

Joel Ohm

Background, 111

Risk factor review, 111

Diagnosis, 115

Management, 117

Follow-up and secondary prevention, 119

Further investigations to consider, 119

Rare underlying causes, 120

Continued follow-up, 121

17.1 Cerebral venous thrombosis, 122

Michael V. Mazya and Joel Ohm

Background, 122

Causes and risk factors, 122

Diagnosis, 122

Management, 123

17.2 Splanchnic vein thrombosis, 125

Maria Magnusson and Per Stål

Background, 125

Diagnosis, 125

Management, 126

18 Specific issues of hemostasis in children – focus on thrombosis, 128

Tony Frisk

Background, 128

Symptoms and signs, 129

Diagnosis, 129

Management, 129

Treatment, 129

Heparin-induced thrombocytopenia (HIT), 132

Percutaneous catheter-directed therapies, 132

Systemic thrombolysis, 132

Catheter-related thrombosis, 133

Cerebral sinus venous thrombosis, 133

Arterial thrombosis, 133

Arterial ischemic stroke (AIS), 134

19 Thrombotic issues with artificial surfaces, 135

Lars Mikael Broman and Moa Simonsson

Prosthetic heart valves, 135

ECMO, 137

20 Treatment and prevention of venous thromboembolism, 142

Margareta Holmström and Joel Ohm

Acute phase therapies, 142

Pharmacological maintenance treatment, 146

Nonpharmacological maintenance treatment, 150

Primary prevention, 151

21 Prevention of cardioembolism in atrial fibrillation, 153

Michael V. Mazya and Moa Simonsson

Background, 153

Assessment of cardioembolic risk, 153

Assessment of bleeding risk, 153

Clinical implications of risk assessment, 154

Stroke prevention in atrial fibrillation, 154

Combination therapy (oral anticoagulant + antiplatelet therapy), 155

Cardioversion, 155

Left atrial appendix closure, 155

Timing of OAC initiation after ischemic stroke and TIA, 156

Resuming oral anticoagulation after intracerebral hemorrhage, 156

22 Laboratory investigation of anticoagulant treatment, 157

Jovan P. Antovic and Maria Farm

Warfarin (PT [INR]), 157

Direct oral anticoagulants (anti-factor IIa and anti-factor Xa), 157

Low molecular weight heparin (anti-factor Xa), 158

Unfractionated heparin (APTT, anti-factor Xa), 158

Argatroban (anti-factor IIa/direct thrombin inhibition, dTT), 159

ARTERIAL THROMBOSIS

23 Ischemic heart disease, 163

Moa Simonsson

Background, 163

Acute coronary syndrome (ACS), 163

Chronic coronary syndrome (CCS), 166

24 Ischemic stroke and transient ischemic attack (TIA), 168

Michael V. Mazya

Background, 168

Causes and risk factors, 168

Diagnosis, 168

Management, 168

25 Peripheral artery disease, 173

Carl Magnus Wahlgren

Background, 173

Antithrombotic therapy for cardiovascular prevention in PAD, 173

Perioperative antithrombotic therapy in PAD, 174

Antithrombotic therapy after endovascular intervention for lower extremity artery disease, 174

Antithrombotic therapy after bypass surgery for lower extremity artery disease, 174

26 Antiplatelet treatment, 176

Michael V. Mazya and Moa Simonsson

Background, 176

Cyclooxygenase-1 inhibitor, 176

Oral P2Y12 inhibitors, 176

Intravenous P2Y12 inhibitor, 177

Glycoprotein IIb/IIIa blocker, 177

27 Laboratory investigation of antiplatelet treatment, 178

Charlotte Gran and Moa Simonsson

Background, 178

VerifyNow, 178

Multiplate, 179

Overall limitations of platelet function tests, 180

PART 4: SPECIAL HEMOSTASIS

28 Hemostasis in rheumatological and systemic disease, 183

Aleksandra Antovic and Francesca Faustini

Introduction, 183

Spondylarthritis and psoriatic arthritis, 184

Systemic autoimmune rheumatic diseases, 184

Management, 185

Antiphospholipid syndrome, 186

29 Hemostasis in COVID-19, 189

Maria Magnusson and Anna Sjöström

Background, 189

Diagnosis, 190

Management, 190

Pediatric aspects including MIS-C, 191

30 Hemostasis in liver disease, 192

Maria Magnusson and Per Stål

Background, 192

Laboratory tests, 192

Management in special situations, 193

31 Hemostasis in kidney disease, 195

Peter Bárány and Maria Magnusson

Background, 195

Bleeding in kidney disease, 195

Thrombosis in kidney disease, 197

32 Hemostasis in obstetrics and gynecology, 199

Roza Chaireti and Maria Sennström

Heavy menstrual bleeding, 199

Bleeding disorders in obstetrics, inherited and aquired, 200

Contraception, hormonal replacement therapy, and risk of thrombosis, 204

Thromboprophylaxis in obstetrics, including risk assessment, 206

Thromboembolism in obstetrics including acute management, 214

33 Hemostasis in cancer, 219

Roza Chaireti, Tony Frisk, Charlotte Gran, and Susanna Ranta

Cancer-associated thrombosis, 219

Hemostasis in children’s malignancy, 221

34 Emergency conditions associated with hemostatic abnormalities, 224

Jovan P. Antovic, Margareta Holmström, and Cecilia Karlström

Disseminated intravascular coagulation, 224

Heparin-induced thrombocytopenia, 226

Thrombotic microangiopathies, 228

Index, 230

Jovan P. Antovic, MD, PhD, Professor in coagulation and clinical chemistry and Senior Consultant, Department of Clinical Chemistry, Karolinska University Hospital and Department of Molecular Medicine and Surgery, Coagulation Research, Karolinska Institutet, Stockholm, Sweden.

Maria Magnusson, MD, PhD, Head of the Department of Hematology and Senior Consultant in pediatric and adult coagulation, Karolinska University Hospital. Affiliated researcher, Department of Molecular Medicine & Surgery and Division of Pediatrics, Department of Clinical Science, Intervention & Technology, Karolinska Institutet.

New edition of a concise, comprehensive, easy-to-read pocket book distilling the most clinically up-to-date information on thrombotic and bleeding disorders

In a concise manner, Blombäck’s Essential Guide to Blood Coagulation distills the most clinically up-to-date information on thrombotic and bleeding disorders, including the latest treatment strategies for key conditions and diseases. This book covers both the stable and the acute stages of hereditary and acquired bleeding and thrombotic disorders in adults, children, and geriatric patients. It also highlights the laboratory tests that can aid in diagnosing and identifying the cause of bleeding and/or thrombosis in a patient.

The book is organized into four main sections, each featuring numerous updates in this new edition. The first part introduces the reader to laboratory hemostasis and hemostasis physiology. The second part covers bleeding disorders, including a new chapter on bleeding disorders and aging. The third part discusses thromboembolic disorders, with a new chapter on extracorporeal membrane oxygenation (ECMO). The final section addresses special issues in hemostasis, with new chapters on hemostasis in renal disease, liver disorders, and cancer, as well as updated information on thrombotic thrombocytopenic purpura.

This edition of Blombäck’s Essential Guide to Blood Coagulation also includes updates on:

• Hemostasis in COVID-19 and vaccine-induced thrombotic thrombocytopenia (VITT), a rare condition observed during pandemic
• Critical bleeding in different clinical situations and hemostasis in obstetrics and gynecology
• Platelet disorders, and rare coagulation factor deficiencies, cerebral vein and splanchnic vein thrombosis
• The greater paradigm shift in the treatment of hereditary bleeding disorders since the last edition was published
• Laboratory issues in hemostasis, including nomenclature and sampling
• Specific issues of hemostasis in children

Compact enough to fit into a coat pocket, Blombäck’s Essential Guide to Blood Coagulation is a valuable guide for all those treating patients to expand their knowledge of hemostatic disorders. This text fills a void for the residents, fellow doctors, and practicing physicians who need rapid access to up-to-date information.