Hemophilia and Hemostasis – A Case–Based Approach to Management

Alice D. Ma, University of North Carolina, Chapel Hill, NC, USA. Miguel A. Escobar, University of Texas Health Science Center at Houston, Director, Gulf States Hemophilia and Thrombophilia Center, Houston, TX, USA. Harold Roberts, MD, ex-Senior Associate Editor of the Journal of Thrombosis and Hemostasis 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, 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 Hemostasis. Excellence in laboratory research and patient care has 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 Hemostasis. 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.

List of Contributors Foreword SECTION 1: HAEMOPHILIA A AND HAEMOPHILIA B General Overview The hemophilic ankle: an update The haemophilic knee: An update Haemophilia with Inhibitors Inhibitor patient requiring high dose therapy with rVIIa as well as sequential therapy with FEIBA. Prophylactic therapy in a patient with a high titer inhibitor Immune Tolerance Induction Monitoring during ITI FIX inhibitors Severe Hemophilia B with high response inhibitor and anaphylactic reaction to factor IX Inhibitor patient and dental surgery Haemophilic treatment for procedures DVT prophylaxis in patients with hemophilia A undergoing orthopedic surgery Prostate Surgery and Hemophilia Mild Hemophilia and Intraocular Injections Endoscopy/colonoscopy and Hemophilia Dialysis and Hemophilia Circumcision PK Studies prior to Orthopedic Surgery Compartment Syndrome Successful eradication of factor VIII inhibitor in patient with mild Hemophilia A prior to hemipelvectomy for extensive hemophilic pseudotumor Coronary artery disease and hemophilia Valve Replacement and Hemophilia Treatment for other conditions Thyroid biopsy and Hemophilia Atrial Fibrillation and bleeding disorders Chronic Upper GI bleeding and hemophilia Hematuria Other issues in haemophilia care Reproductive Options for Hemophilia A Carriers Mild Hemophilia A with Discrepant FVIII activity levels Compund Diagnoses Hemophilia A with tuberous sclerosis and CNS bleed Familial Risk Assessment for Individuals with Hemophilia A and von Willebrand Disease Hemophilia A and HHT SECTION 2: VON WILLEBRAND DISEASE Management during procedures Type I VWD Tonsillectomy VWD and Dental surgery VWD and GI surgery VWD and Obstetric/Gynecologic Procedures Rare forms of Von Willebrand Disease Type 2A VWD and recurrent GI bleeding Type 2B VWD and Thoracic Surgery Von Willebrand disease 2N SECTION 3: OTHER BLEEDING DISORDERS Prothrombin deficiency Factor V deficiency Factor VII deficiency FX deficiency Factor XI deficiency Factor XIII deficiency Combined factor V and factor VIII deficiency 1, 2 Glanzmann Thrombaesthenia Gardner-Diamond syndrome and VWD Qualitative Platelet Disorder-QPD SECTION 4: ACQUIRED BLEEDING DISORDERS Acquired FVIII inhibitor and B cell neoplasm FVIII inhibitor and lupus inhibitor Acquired VWD A woman with bleeding gums Bleeding after cardiac surgery Bleeding in a dialysis patient A woman with anemia and hematuria Scalp bleeding in an older gentleman Hyperfibrinolysis SECTION 5: THROMBOTIC DISORDERS Heparin induced thrombocytopenia with thrombosis Heparin Skin Necrosis Warfarin skin necrosis Thoracic outlet syndrome 3, 4 Antithrombin Deficiency May-Thurner syndrome Thrombosis in a liver transplant patient Combined thrombophilia Index