Haemophilia and Haemostasis (eBook)

Harold Roberts, MD, ex-Senior Associate Editor of the Journal of Thrombosis and Haemostasis and Chairman, Division of Hematology/Oncology, University of North Carolina at Chapel Hill, Chapel Hill North Carolina, USA. Dr Roberts, a Sarah Graham Kenan Professor of Medicine and Pathology at the UNC-Chapel Hill School of Medicine, was born in Four Oaks and grew up in Sanford. He earned his undergraduate and medical degrees from UNC-Chapel Hill, joined the faculty in 1962 and has served as chief of the division of hematology and director of the UNC-Chapel Hill Comprehensive Hemophilia Diagnostic and Treatment Center. He was also the founding director of the school's Center for Thrombosis and Hemostasis, which conducts research on blood clotting disorders related to cardiovascular disease and on bleeding disorders such as hemophilia. He has served on the editorial boards of Current Opinion in Hematology and Hematologic Pathology, as well as being senior associate editor of Thrombosis and Haemostasis. Excellence in laboratory research and patient care earned Dr. Roberts numerous awards: the French International Prize for Research on Hemophilia, the National Hemophilia Foundation's Kenneth M. Brinkhous Award for Excellence in Clinical Research, and the Robert P. Grant Medal, the highest honor given by the International Society on Thrombosis and Haemostasis. The citation accompanying this award lauded him as "one of the pioneers in the field of coagulation as well as being a leader in the Chapel Hill group which has brought so much to our present understanding of the subtleties in the mechanisms of coagulation. His contributions to our field have had a major impact, especially his outstanding work on the genetic basis of hemophilia." In spring of 2000, Dr. Roberts received the UNC Medical Alumni Association's Distinguished Faculty Award and an honorary doctor of medicine degree from Lund University in Sweden. Daniel Carrizosa, MD, Consultant Hematologist, Division of Hematology/Oncology, University of North Carolina at Chapel Hill, Chapel Hill North Carolina, USA. Alice Ma, MD, Division of Hematology/Oncology, University of North Carolina at Chapel Hill, Chapel Hill North Carolina, USA.

Haemophilia and Haemostasis: A Case-based Approach to Management 1
Contents 7
List of Haemostasis Forum Editors 11
List of Contributors 17
Preface 21
1 Haemophilia A and Haemophilia B 23
General Overview 25
Haemophilia and Immune Tolerance Therapy 25
The Haemophilic Ankle: An Update 27
The Haemophilic Knee: An Update 37
Issues with Complicated Diagnoses 45
Combined Haemophilia A and B Carrier 45
A Complex Case of Haemophilia with HIV and Hepatitis C 47
A Case of Haemophilia B, Mild VWD, and a Factor IX Inhibitor 51
Premature Infant with Haemophilia B 55
Haemophilic Carriers 57
Haemophilic Carriers and Delivery 57
Mild Haemophilia in Women 59
Treatment of the Pregnant Haemophiliac 63
Anticoagulation and Haemophilia 65
Anticoagulation for Atrial Fibrillation in a Haemophiliac 65
Anticoagulation for a Cardiac Valve in a Haemophiliac 69
Cardiac Catheterization in a Haemophiliac 71
Anticoagulation for a DVT in a Haemophiliac 73
Treatment of Haemophiliacs 77
DDAVP for Treatment of Mild Haemophilia during Surgery 77
Haemophilia and Haemodialysis 79
Haemophilia and Hepatitis C Treatment 81
Haemophilia and Physical Therapy 85
Haemophilia and Renal Bleeds 87
Haemophilia and Scuba Diving 89
Haemophilia and Ventricular Septal Defect Repair 91
Haemophilia with Hepatitis C and Recurrent Bleeding 95
Isotretinoin in Haemophilia 97
Laser Eye Surgery in a Haemophiliac 99
Managing Haemophilic Pseudotumours 101
Continuous NovoSeven: Pros and Cons 103
Complications of Treatment 107
Haemophilia B and Immune Tolerance with Anaphylaxis 107
rFVIIa (NovoSeven) 109
Thrombosis in PCCs vs. APCCs 113
2 Von Willebrand Disease 115
Epidurals and VWD 117
Anticoagulation for a Cardiac Valve in a Patient with VWD Type 1 119
VWD Type 2A and Pregnancy 121
VWD Type 2B and Pregnancy 123
VWD Type 2B vs. Platelet Type 125
Prophylaxis in VWD Type 3 127
Platelet Type VWD 129
3 Factor Deficiencies 131
Combined Factor V and VIII Deficiency 133
DVT Prophylaxis in FVII Deficiency 135
Menorrhagia in Factor VII Deficiency 137
Anticoagulation for Atrial Fibrillation in FX Deficiency 141
Management of Factor X Deficiency 143
Anticoagulation for Atrial Fibrillation in a Patient with Factor XI Deficiency 145
Factor XI Deficiency and Surgery 147
Prophylaxis for Patients with Factor XIII Deficiency and Intracranial Bleeding 149
4 Rare Platelet and Coagulation Disorders 151
Coagulation Disorders 153
Afibrinogenaemia 153
Dysfibrinogenaemia 157
Hypofibrinogenaemia 161
Gardner-Diamond Syndrome 163
Hereditary Haemorrhagic Telangiectasia 167
Hereditary Vitamin-K-Dependent Coagulation Factors Deficiency and Pregnancy 173
Platelet Disorders 175
Glanzmann's Thrombasthaenia and Gastrointestinal Angiodysplasia 175
Glanzmann's Thrombasthaenia and Pregnancy 179
rVIIa (NovoSeven®) and Wiskott-Aldrich Syndrome 181
5 Acquired Bleeding Diatheses 183
Acquired Haemophilia and Second Pregnancy 185
Developing a Factor IX Inhibitor 187
End-Stage Liver Disease and Surgery 189
Treatment for Acute DIC 191
6 Miscellaneous Questions 195
Bleeding Time vs. PFA-100 197
Cocaine and DDAVP 199
Hyponatraemia and DDAVP 201
The Use of the INR 203
Selective Serotonin Reuptake Inhibitors and Clotting Disturbances 207
Work-up for Children with Intracranial Bleeding 211
7 Thrombotic Disorders 215
Are Asians Genetically Different from Westerners When it Comes to VTE? 217
Treatment of Antithrombin Deficiency 223
Anticardiolipin Antibody Questions 225
Paediatric Antiphospholipid Syndrome and Recurrent Thrombosis 227
Anticoagulation for Deep Venous Thrombosis in the Presence of an Intracranial Haemorrhage 231
Clinical Probability Assessment for Thromboembolic Disease 235
D-dimer in Thromboembolic Disease 237
Progestins and Thrombosis 239
Unknown Thrombophilia and Surgery 243
Index 245