Hemophilia and Hemostasis

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