Rezan A. Kadir, Professor in the Department of Obstetrics and Gynaecology and Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free Foundation Hospital and Institute for Women's Health, University College London, London, UK. Paula D. James, Professor in the Department of Medicine, Queen's University, Kingston, Canada. Christine A. Lee, Emeritus Professor of Hemophilia, University College London, London, UK.

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• Preface to Second Edition ixPreface to First Edition xiList of Contributors xiii1 Hematological Assessment of a Patient with an Inherited Bleeding Disorder 1 Sue Pavord and Henna Wong1.1 Introduction 11.2 Normal Hemostasis 11.3 Defects of Hemostasis 51.4 Clinical Presentation of Bleeding 51.5 Diagnosis 61.6 Approach to a Female with a Bleeding History 61.7 Summary 10References 112 Bleeding Assessment Tools 13Sarah H. O’Brien and Paula D. James2.1 Introduction 132.2 Evolution of Vicenza‐Based Bleeding Assessment Tools 132.3 Women’s Studies Using Vincenza‐Based Bleeding Tools 222.4 Tools to Evaluate Menstrual Blood Loss 232.5 Young Women’s Hematology and Bleeding Assessment Tools 252.6 Clinical Utility of Bleeding Assessment Tools 252.7 Conclusion 26References 273 Physiology of Menstruation 29Jane J. Reavey, Jacqueline A. Maybin, and Hilary O.D. Critchley3.1 Introduction 293.2 Normal Menstruation 293.3 Neuroendocrine Hormones 303.4 Menarche 313.5 Ovarian Follicle Development and Endocrine Function 313.6 Endometrium 333.7 Secretory Phase 343.8 Menstruation 353.9 Control of Bleeding 373.10 Endometrial Repair 383.11 Proliferative Phase 403.12 Summary 40References 414 Gynecology 45Joanna S. Davies and Rezan A. Kadir4.1 Introduction 454.2 Heavy Menstrual Bleeding 484.3 Management of Acute Episodes of Heavy Menstrual Bleeding 584.4 Ovulation Bleeding 594.5 Endometriosis 594.6 Other Gynecological Conditions 604.7 Conclusion 61References 615 Carriers of Hemophilia A and Hemophilia B 65Roseline d’Oiron5.1 Inheritance 655.2 Screening for the Genetic Status of Carriers of Hemophilia 655.3 Confusion Between Genetic and Coagulation Testing 665.4 When to Perform Genetic Testing 675.5 What Reasons might Contribute to Delayed Genetic Diagnosis of Carriership? 685.6 Bleeding Disorders in Carriers of Hemophilia 685.7 Quality of Life of Carriers of Hemophilia 735.8 Carriers of Hemophilia A and B and Pregnancy 735.9 How to Improve Care for Carriers of Hemophilia 77References 796 Von Willebrand Disease 83Carolyn M. Millar6.1 Introduction 836.2 Structure and Function of Von Willebrand Factor 836.3 Von Willebrand Factor Levels and Prevalence of Von Willebrand Disease 846.4 VWF Levels, the Menstrual Cycle and Pregnancy 846.5 Von Willebrand Disease Classification and Inheritance 856.6 Clinical Presentation 876.7 Menorrhagia and Postpartum Hemorrhage 876.8 Diagnosis and Laboratory Testing 886.9 Management of Von Willebrand Disease 906.10 Management of Gynecological Bleeding 916.11 Obstetric Management of Von Willebrand Disease 926.12 Neonatal Management 946.13 Cases 94References 967 Factor XI Deficiency 101Bethan Myers and Rezan A. Kadir7.1 Factor XI Structure and Function in Coagulation 1017.2 Incidence and Inheritance of Factor XI Deficiency 1017.3 Bleeding Manifestations and Diagnosis of Factor XI Deficiency 1037.4 Factor XI Deficiency and Gynecological Issues 1037.5 Factor XI Deficiency and Pregnancy 1057.6 Neuroaxial Analgesia and Anesthesia 1087.7 Management of Postpartum Period 1087.8 Treatment Options 1097.9 Neonatal Bleeding 1117.10 Conclusions and Recommendations 111References 1128 Rare Bleeding Disorders 117Danijela Mikovic, Marzia Menegatti, and Flora Peyvandi8.1 Introduction 1178.2 Clinical Symptoms 1188.3 Gynecological and Obstetrical Manifestations in Women with Rare Bleeding Disorders 1198.4 Laboratory Diagnosis 1228.5 Differential Diagnosis in Women with Menorrhagia 1238.6 Treatment 1248.7 Conclusion 128References 1299 Inherited Platelet Defects 133Mike Makris and Clare Samuelson9.1 Introduction 1339.2 Normal Platelet Function 1339.3 Presentation 1369.4 Investigation 1379.5 Syndromic and Non‐Syndromic Inherited Platelet Disorders with Recognized Causative Genes 1439.6 Prenatal Diagnosis 1439.7 Management 1449.8 Future Directions 1479.9 Case Histories 1479.10 Conclusion 148References 14910 Genetic and Laboratory Diagnosis 153Anne C. Goodeve10.1 Introduction 15310.2 Phenotypic Analysis of Hemophilia A 15410.3 Phenotypic Analysis of Hemophilia B 15610.4 Phenotypic Analysis of von Willebrand Disease 15710.5 Phenotypic Analysis of Inherited Bleeding Disorders 15710.6 Genetic Analysis of Hemophilia A 16010.7 Genetic Analysis of Hemophilia B 16210.8 Genetic Analysis of von Willebrand Disease 16310.9 Guidelines 16410.10 Summary 164References 16411 Antenatal Diagnosis 167Rezan A. Kadir, Irena Hudecova, and Claudia Chi11.1 Introduction 16711.2 Genetic Counseling 16711.3 Prenatal Diagnosis 17011.4 Prenatal Diagnosis of Hemophilia 17711.5 Prenatal Diagnosis of von Willebrand Disease 18011.6 Prenatal Diagnosis of Rare Bleeding Disorders 18011.7 Preimplantation Genetic Diagnosis 18111.8 Views about and Experiences of Prenatal Diagnosis of Women in Families Affected with Inherited Bleeding Disorders 18311.9 Termination of Pregnancy 185References 18612 Analgesia and Anesthesia for Pregnant Women with Inherited Bleeding Disorders 191Anne‐Sophie Bouthors, Adrian England, and Rezan A. Kadir12.1 Introduction 19112.2 Non‐Pharmacological Methods 19212.3 Pharmacological Methods 19212.4 Conclusion 201References 20213 The Newborn 205Manuel Carcao and Vanessa Bouskill13.1 Introduction 20513.2 Developmental Hemostasis 20513.3 Laboratory Hemostatic Evaluation of the Neonate 20813.4 When to Suspect a Congenital Bleeding Disorder in a Newborn 20913.5 Congenital Bleeding Disorders and Their Presentation in Newborns 21313.6 Management of Bleeding in Neonates with Congenital Bleeding Disorders 21813.7 Conclusion 221References 22214 Women with Inherited Bleeding Disorders in Different Cultural Settings 225Tahira Zafar, Jameela Sathar, Ali T. Taher, Fadi G. Mirza, and Christine A. Lee14.1 Introduction 22514.2 Pakistan 22514.3 Malaysia 22714.4 Lebanon 22914.5 Discussion 231References 232Index 235

Rezan A. Kadir, Professor in the Department of Obstetrics and Gynaecology and Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free Foundation Hospital and Institute for Women's Health, University College London, London, UK. Paula D. James, Professor in the Department of Medicine, Queen's University, Kingston, Canada. Christine A. Lee, Emeritus Professor of Hemophilia, University College London, London, UK.

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• Preface to Second Edition ixPreface to First Edition xiList of Contributors xiii1 Hematological Assessment of a Patient with an Inherited Bleeding Disorder 1 Sue Pavord and Henna Wong1.1 Introduction 11.2 Normal Hemostasis 11.3 Defects of Hemostasis 51.4 Clinical Presentation of Bleeding 51.5 Diagnosis 61.6 Approach to a Female with a Bleeding History 61.7 Summary 10References 112 Bleeding Assessment Tools 13Sarah H. O’Brien and Paula D. James2.1 Introduction 132.2 Evolution of Vicenza‐Based Bleeding Assessment Tools 132.3 Women’s Studies Using Vincenza‐Based Bleeding Tools 222.4 Tools to Evaluate Menstrual Blood Loss 232.5 Young Women’s Hematology and Bleeding Assessment Tools 252.6 Clinical Utility of Bleeding Assessment Tools 252.7 Conclusion 26References 273 Physiology of Menstruation 29Jane J. Reavey, Jacqueline A. Maybin, and Hilary O.D. Critchley3.1 Introduction 293.2 Normal Menstruation 293.3 Neuroendocrine Hormones 303.4 Menarche 313.5 Ovarian Follicle Development and Endocrine Function 313.6 Endometrium 333.7 Secretory Phase 343.8 Menstruation 353.9 Control of Bleeding 373.10 Endometrial Repair 383.11 Proliferative Phase 403.12 Summary 40References 414 Gynecology 45Joanna S. Davies and Rezan A. Kadir4.1 Introduction 454.2 Heavy Menstrual Bleeding 484.3 Management of Acute Episodes of Heavy Menstrual Bleeding 584.4 Ovulation Bleeding 594.5 Endometriosis 594.6 Other Gynecological Conditions 604.7 Conclusion 61References 615 Carriers of Hemophilia A and Hemophilia B 65Roseline d’Oiron5.1 Inheritance 655.2 Screening for the Genetic Status of Carriers of Hemophilia 655.3 Confusion Between Genetic and Coagulation Testing 665.4 When to Perform Genetic Testing 675.5 What Reasons might Contribute to Delayed Genetic Diagnosis of Carriership? 685.6 Bleeding Disorders in Carriers of Hemophilia 685.7 Quality of Life of Carriers of Hemophilia 735.8 Carriers of Hemophilia A and B and Pregnancy 735.9 How to Improve Care for Carriers of Hemophilia 77References 796 Von Willebrand Disease 83Carolyn M. Millar6.1 Introduction 836.2 Structure and Function of Von Willebrand Factor 836.3 Von Willebrand Factor Levels and Prevalence of Von Willebrand Disease 846.4 VWF Levels, the Menstrual Cycle and Pregnancy 846.5 Von Willebrand Disease Classification and Inheritance 856.6 Clinical Presentation 876.7 Menorrhagia and Postpartum Hemorrhage 876.8 Diagnosis and Laboratory Testing 886.9 Management of Von Willebrand Disease 906.10 Management of Gynecological Bleeding 916.11 Obstetric Management of Von Willebrand Disease 926.12 Neonatal Management 946.13 Cases 94References 967 Factor XI Deficiency 101Bethan Myers and Rezan A. Kadir7.1 Factor XI Structure and Function in Coagulation 1017.2 Incidence and Inheritance of Factor XI Deficiency 1017.3 Bleeding Manifestations and Diagnosis of Factor XI Deficiency 1037.4 Factor XI Deficiency and Gynecological Issues 1037.5 Factor XI Deficiency and Pregnancy 1057.6 Neuroaxial Analgesia and Anesthesia 1087.7 Management of Postpartum Period 1087.8 Treatment Options 1097.9 Neonatal Bleeding 1117.10 Conclusions and Recommendations 111References 1128 Rare Bleeding Disorders 117Danijela Mikovic, Marzia Menegatti, and Flora Peyvandi8.1 Introduction 1178.2 Clinical Symptoms 1188.3 Gynecological and Obstetrical Manifestations in Women with Rare Bleeding Disorders 1198.4 Laboratory Diagnosis 1228.5 Differential Diagnosis in Women with Menorrhagia 1238.6 Treatment 1248.7 Conclusion 128References 1299 Inherited Platelet Defects 133Mike Makris and Clare Samuelson9.1 Introduction 1339.2 Normal Platelet Function 1339.3 Presentation 1369.4 Investigation 1379.5 Syndromic and Non‐Syndromic Inherited Platelet Disorders with Recognized Causative Genes 1439.6 Prenatal Diagnosis 1439.7 Management 1449.8 Future Directions 1479.9 Case Histories 1479.10 Conclusion 148References 14910 Genetic and Laboratory Diagnosis 153Anne C. Goodeve10.1 Introduction 15310.2 Phenotypic Analysis of Hemophilia A 15410.3 Phenotypic Analysis of Hemophilia B 15610.4 Phenotypic Analysis of von Willebrand Disease 15710.5 Phenotypic Analysis of Inherited Bleeding Disorders 15710.6 Genetic Analysis of Hemophilia A 16010.7 Genetic Analysis of Hemophilia B 16210.8 Genetic Analysis of von Willebrand Disease 16310.9 Guidelines 16410.10 Summary 164References 16411 Antenatal Diagnosis 167Rezan A. Kadir, Irena Hudecova, and Claudia Chi11.1 Introduction 16711.2 Genetic Counseling 16711.3 Prenatal Diagnosis 17011.4 Prenatal Diagnosis of Hemophilia 17711.5 Prenatal Diagnosis of von Willebrand Disease 18011.6 Prenatal Diagnosis of Rare Bleeding Disorders 18011.7 Preimplantation Genetic Diagnosis 18111.8 Views about and Experiences of Prenatal Diagnosis of Women in Families Affected with Inherited Bleeding Disorders 18311.9 Termination of Pregnancy 185References 18612 Analgesia and Anesthesia for Pregnant Women with Inherited Bleeding Disorders 191Anne‐Sophie Bouthors, Adrian England, and Rezan A. Kadir12.1 Introduction 19112.2 Non‐Pharmacological Methods 19212.3 Pharmacological Methods 19212.4 Conclusion 201References 20213 The Newborn 205Manuel Carcao and Vanessa Bouskill13.1 Introduction 20513.2 Developmental Hemostasis 20513.3 Laboratory Hemostatic Evaluation of the Neonate 20813.4 When to Suspect a Congenital Bleeding Disorder in a Newborn 20913.5 Congenital Bleeding Disorders and Their Presentation in Newborns 21313.6 Management of Bleeding in Neonates with Congenital Bleeding Disorders 21813.7 Conclusion 221References 22214 Women with Inherited Bleeding Disorders in Different Cultural Settings 225Tahira Zafar, Jameela Sathar, Ali T. Taher, Fadi G. Mirza, and Christine A. Lee14.1 Introduction 22514.2 Pakistan 22514.3 Malaysia 22714.4 Lebanon 22914.5 Discussion 231References 232Index 235

Rezan A. Kadir, Professor in the Department of Obstetrics and Gynaecology and Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free Foundation Hospital and Institute for Women's Health, University College London, London, UK. Paula D. James, Professor in the Department of Medicine, Queen's University, Kingston, Canada. Christine A. Lee, Emeritus Professor of Hemophilia, University College London, London, UK.

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• Preface to Second Edition ixPreface to First Edition xiList of Contributors xiii1 Hematological Assessment of a Patient with an Inherited Bleeding Disorder 1 Sue Pavord and Henna Wong1.1 Introduction 11.2 Normal Hemostasis 11.3 Defects of Hemostasis 51.4 Clinical Presentation of Bleeding 51.5 Diagnosis 61.6 Approach to a Female with a Bleeding History 61.7 Summary 10References 112 Bleeding Assessment Tools 13Sarah H. O’Brien and Paula D. James2.1 Introduction 132.2 Evolution of Vicenza‐Based Bleeding Assessment Tools 132.3 Women’s Studies Using Vincenza‐Based Bleeding Tools 222.4 Tools to Evaluate Menstrual Blood Loss 232.5 Young Women’s Hematology and Bleeding Assessment Tools 252.6 Clinical Utility of Bleeding Assessment Tools 252.7 Conclusion 26References 273 Physiology of Menstruation 29Jane J. Reavey, Jacqueline A. Maybin, and Hilary O.D. Critchley3.1 Introduction 293.2 Normal Menstruation 293.3 Neuroendocrine Hormones 303.4 Menarche 313.5 Ovarian Follicle Development and Endocrine Function 313.6 Endometrium 333.7 Secretory Phase 343.8 Menstruation 353.9 Control of Bleeding 373.10 Endometrial Repair 383.11 Proliferative Phase 403.12 Summary 40References 414 Gynecology 45Joanna S. Davies and Rezan A. Kadir4.1 Introduction 454.2 Heavy Menstrual Bleeding 484.3 Management of Acute Episodes of Heavy Menstrual Bleeding 584.4 Ovulation Bleeding 594.5 Endometriosis 594.6 Other Gynecological Conditions 604.7 Conclusion 61References 615 Carriers of Hemophilia A and Hemophilia B 65Roseline d’Oiron5.1 Inheritance 655.2 Screening for the Genetic Status of Carriers of Hemophilia 655.3 Confusion Between Genetic and Coagulation Testing 665.4 When to Perform Genetic Testing 675.5 What Reasons might Contribute to Delayed Genetic Diagnosis of Carriership? 685.6 Bleeding Disorders in Carriers of Hemophilia 685.7 Quality of Life of Carriers of Hemophilia 735.8 Carriers of Hemophilia A and B and Pregnancy 735.9 How to Improve Care for Carriers of Hemophilia 77References 796 Von Willebrand Disease 83Carolyn M. Millar6.1 Introduction 836.2 Structure and Function of Von Willebrand Factor 836.3 Von Willebrand Factor Levels and Prevalence of Von Willebrand Disease 846.4 VWF Levels, the Menstrual Cycle and Pregnancy 846.5 Von Willebrand Disease Classification and Inheritance 856.6 Clinical Presentation 876.7 Menorrhagia and Postpartum Hemorrhage 876.8 Diagnosis and Laboratory Testing 886.9 Management of Von Willebrand Disease 906.10 Management of Gynecological Bleeding 916.11 Obstetric Management of Von Willebrand Disease 926.12 Neonatal Management 946.13 Cases 94References 967 Factor XI Deficiency 101Bethan Myers and Rezan A. Kadir7.1 Factor XI Structure and Function in Coagulation 1017.2 Incidence and Inheritance of Factor XI Deficiency 1017.3 Bleeding Manifestations and Diagnosis of Factor XI Deficiency 1037.4 Factor XI Deficiency and Gynecological Issues 1037.5 Factor XI Deficiency and Pregnancy 1057.6 Neuroaxial Analgesia and Anesthesia 1087.7 Management of Postpartum Period 1087.8 Treatment Options 1097.9 Neonatal Bleeding 1117.10 Conclusions and Recommendations 111References 1128 Rare Bleeding Disorders 117Danijela Mikovic, Marzia Menegatti, and Flora Peyvandi8.1 Introduction 1178.2 Clinical Symptoms 1188.3 Gynecological and Obstetrical Manifestations in Women with Rare Bleeding Disorders 1198.4 Laboratory Diagnosis 1228.5 Differential Diagnosis in Women with Menorrhagia 1238.6 Treatment 1248.7 Conclusion 128References 1299 Inherited Platelet Defects 133Mike Makris and Clare Samuelson9.1 Introduction 1339.2 Normal Platelet Function 1339.3 Presentation 1369.4 Investigation 1379.5 Syndromic and Non‐Syndromic Inherited Platelet Disorders with Recognized Causative Genes 1439.6 Prenatal Diagnosis 1439.7 Management 1449.8 Future Directions 1479.9 Case Histories 1479.10 Conclusion 148References 14910 Genetic and Laboratory Diagnosis 153Anne C. Goodeve10.1 Introduction 15310.2 Phenotypic Analysis of Hemophilia A 15410.3 Phenotypic Analysis of Hemophilia B 15610.4 Phenotypic Analysis of von Willebrand Disease 15710.5 Phenotypic Analysis of Inherited Bleeding Disorders 15710.6 Genetic Analysis of Hemophilia A 16010.7 Genetic Analysis of Hemophilia B 16210.8 Genetic Analysis of von Willebrand Disease 16310.9 Guidelines 16410.10 Summary 164References 16411 Antenatal Diagnosis 167Rezan A. Kadir, Irena Hudecova, and Claudia Chi11.1 Introduction 16711.2 Genetic Counseling 16711.3 Prenatal Diagnosis 17011.4 Prenatal Diagnosis of Hemophilia 17711.5 Prenatal Diagnosis of von Willebrand Disease 18011.6 Prenatal Diagnosis of Rare Bleeding Disorders 18011.7 Preimplantation Genetic Diagnosis 18111.8 Views about and Experiences of Prenatal Diagnosis of Women in Families Affected with Inherited Bleeding Disorders 18311.9 Termination of Pregnancy 185References 18612 Analgesia and Anesthesia for Pregnant Women with Inherited Bleeding Disorders 191Anne‐Sophie Bouthors, Adrian England, and Rezan A. Kadir12.1 Introduction 19112.2 Non‐Pharmacological Methods 19212.3 Pharmacological Methods 19212.4 Conclusion 201References 20213 The Newborn 205Manuel Carcao and Vanessa Bouskill13.1 Introduction 20513.2 Developmental Hemostasis 20513.3 Laboratory Hemostatic Evaluation of the Neonate 20813.4 When to Suspect a Congenital Bleeding Disorder in a Newborn 20913.5 Congenital Bleeding Disorders and Their Presentation in Newborns 21313.6 Management of Bleeding in Neonates with Congenital Bleeding Disorders 21813.7 Conclusion 221References 22214 Women with Inherited Bleeding Disorders in Different Cultural Settings 225Tahira Zafar, Jameela Sathar, Ali T. Taher, Fadi G. Mirza, and Christine A. Lee14.1 Introduction 22514.2 Pakistan 22514.3 Malaysia 22714.4 Lebanon 22914.5 Discussion 231References 232Index 235