Hemophilia and Hemostasis. a Case-Based Approach to Management

Description

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.

Table of Contents

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