There is a general need amongst healthcare professionals for practical advice on the management of patients with bleeding disorders. This book is an essential resource for all those working in the fields of coagulation, hemostasis and thrombosis. It covers the major cases one might encounter in diagnosing, managing and treating hemophilia and hemostasis. It provides a practical and informative guide to the broad range of topics concerning both bleeding and clotting disorders.
The book is divided into major chapter sections depending on the type of bleeding disorder it fits into. Each chapter includes a brief overview of the disorder covering: history of the disorder; molecular basis of the disorder; class presentation; genetics; current laboratory tests and monitoring. Cases associated with each disorder are presented alongside practical questions and answers from a wide range of contributors. As practice can vary from center to center, controversial areas are clearly marked and discussed throughout.
New to this edition: coverage of the newer techniques; newer treatment modalities; new oral anticoagulants; update on hemophilia management; more on ITP and greater coverage of new cases as suggested by reviewers.
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 theJournal 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 ofCurrent Opinion in Hematology andHematologic Pathology, as well as being Senior Associate Editor ofThrombosis 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 ContributorsForeword
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 WillebrandDisease
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 DisorderQPD
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