Haemophilia and Haemostasis – A Case–based Approach to Management

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.

List of Haemostasis Forum Editors. List of Contributors. Preface. 1 Hemophilia A and Hemophilia B. General Overview. Hemophilia and Immune Tolerance Therapy. The Hemophilic Ankle: An update. The Hemophilic Knee: An update. Issues with Complicated Diagnoses. Combined Hemophilia A and B Carrier. A Complex Case of Hemophilia with HIV and Hepatitis C. A Case of Hemophilia B, Mild VWD and a Factor IX Inhibitor. Premature Infant with Hemophilia B. Hemophilic Carriers. Hemophilic Carriers and Delivery. Mild Hemophilia in Women. Treatment of the Pregnant Hemophiliac. Anticoagulation and Hemophilia. Anticoagulation for Atrial Fibrillation in a Hemophiliac. Anticoagulation for a Cardiac Valve in a Hemophiliac. Cardiac Catheterization in a Hemophiliac. Anticoagulation for a DVT in a Hemophiliac. Treatment of Hemophiliacs. DDAVP for Treatment of Mild Hemophilia during Surgery. Hemophilia and Hemodialysis. Hemophilia and Hepatitis C Treatment. Hemophilia and Physical Therapy. Hemophilia and Renal Bleeds. Hemophilia and SCUBA Diving. Hemophilia and Ventricular Septal Defect Repair. Hemophilia with Hepatitis C and Recurrent Bleeding. Isotretinoin in Hemophilia. Laser Eye Surgery in a Hemophiliac. Managing Hemophilic Pseudotumors. Continuous NovoSeven: Pros and Cons. Complications of Treatment. Hemophilia B and Immune Tolerance with Anaphylaxis. NovoSeven (rFVIIa) and DIC. Thrombosis in Prothrombin Complex Concentrates (PCCs) vs. activated Prothrombin Complex Concentrates (APCCs). 2 Von Willebrand Disease (VWD). Epidurals and VWD. Anticoagulation for a Cardiac Valve in a patient with VWD type 1. VWD type 2a and Pregnancy. VWD type 2b and Pregnancy. VWD type 2b Versus Platelet Type. Prophylaxis in VWD type 3. Platelet Type VWD. 3 Factor Deficiencies. Combined Factor V and VIII Deficiency. DVT Prophylaxis in FVII Deficiency. Menorrhagia in Factor VII Deficiency. Anticoagulation for Atrial Fibrillation in FX Deficiency. Management of Factor X Deficiency. Anticoagulation for Atrial Fibrillation in a patient with Factor XI Deficiency. Factor XI Deficiency and Surgery. Prophylaxis for Patients with Factor XIII Deficiency and Intracranial Bleeding. 4 Rare Platelet and Coagulation Disorders. Coagulation Disorders. Afibrinogenemia. Dysfibrinogenemia. Hypofibrinogenemia. Gardner-Diamond Syndrome. Hereditary Hemorrhagic Telangiectasia (HHT). Hereditary Vitamin K Dependent Coagulation Factors Deficiency and Pregnancy. Platelet Disorders. Glanzmann's Thrombasthenia and Gastrointestinal Angiodysplasia. Glanzmann's Thrombasthenia and Pregnancy. NovoSeven and Wiscott-Aldrich. 5 Acquired Bleeding Diatheses. Acquired Hemophilia and Second Pregnancy. Developing a Factor IX Inhibitor. Acquired Bleeding Diatheses:. End-Stage Liver Disease and Surgery. Treatment for Acute DIC. 6 Miscellaneous Questions. Bleeding Time Versus PFA-100. Cocaine and DDAVP. Hyponatremia and DDAVP. The Use of the INR. Selective Serotonin Reuptake Inhibitors and Clotting Disturbances. Work-up for Children with Intracranial Bleeding. 7 Thrombotic Disorders. Are Asians genetically different from Westerners when it comes to VTE?. Treatment of Antithrombin Deficiency. Anticardiolipin Antibody Questions. Pediatric Antiphospholipid Syndrome and Recurrent Thrombosis. Anticoagulation for Deep Venous Thrombosis in the Presence of an Intracranial Hemorrhage. Clinical Probability Assessment for Thromboembolic Disease. D-dimer in Thromboembolic Disease. Progestins and Thrombosis. Unknown Thrombophilia and Surgery