Hemophilia and Hemostasis E-Book

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 226

Veröffentlichungsjahr: 2012

Beschreibung

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.

Leseprobe

Contents

Contributors

Foreword

PART 1 Hemophilia A and Hemophilia B

SECTION I General Overview

INTRODUCTION 1 The Hemophilic Ankle: An Update

Pathogenesis of synovitis and cartilage damage in hemophilia: experimental studies

Radiosynovectomy

Hemophilic arthropathy of the ankle and subtalar joints

Rehabilitation and physiotherapy

Conclusions

INTRODUCTION 2 The Hemophilic Knee: An Update

Joint aspiration (arthrocentesis)

Conclusions

SECTION II Hemophilia with Inhibitors

CASE STUDY 1 Inhibitor Patient Requiring High Dose Therapy with rVIIa as well as Sequential Therapy with FEIBA

CASE STUDY 2 Prophylactic Therapy in a Patient with a High Titer Inhibitor

CASE STUDY 3 Immune Tolerance Induction

CASE STUDY 4 Monitoring During Immune Tolerance Induction

CASE STUDY 5 Factor IX Inhibitors

CASE STUDY 6 Severe Hemophilia B with High Response Inhibitor and Anaphylactic Reaction to Factor IX

CASE STUDY 7 Inhibitor Patient and Dental Surgery

SECTION III Hemophilic Treatment for Procedures

CASE STUDY 8 Deep Vein Thrombosis Prophylaxis in Patients with Hemophilia A Undergoing Orthopedic Surgery

CASE STUDY 9 Prostate Surgery and Hemophilia

CASE STUDY 10 Mild Hemophilia and Intraocular Injections

CASE STUDY 11 Endoscopy/Colonoscopy and Hemophilia

CASE STUDY 12 Dialysis and Hemophilia

CASE STUDY 13 Circumcision

CASE STUDY 14 Pharmacokinetic Studies for Surgery

CASE STUDY 15 Compartment Syndrome

CASE STUDY 16 Successful Eradication of Factor VIII Inhibitor in Patient with Mild Hemophilia A Prior to Hemipelvectomy for Extensive Hemophilic Pseudotumor

CASE STUDY 17 Coronary Artery Disease and Hemophilia

CASE STUDY 18 Valve Replacement and Hemophilia

SECTION IV Treatment for Other Conditions

CASE STUDY 19 Thyroid Biopsy and Hemophilia

CASE STUDY 20 Atrial Fibrillation and Bleeding Disorders

CASE STUDY 21 Chronic Upper Gastrointestinal Bleeding and Hemophilia

CASE STUDY 22 Hematuria

SECTION V Other Issues in Hemophilia Care

CASE STUDY 23 Reproductive Options for Hemophilia A Carriers

CASE STUDY 24 Mild Hemophilia A with Discrepant FVIII Activity Levels

SECTION VI Compound Diagnoses

CASE STUDY 25 Hemophilia A with Tuberous Sclerosis and CNS Bleed

CASE STUDY 26 Familial Risk Assessment for Individuals with Hemophilia A and von Willebrand Disease

CASE STUDY 27 Hemophilia A and Hereditary Hemorrhagic Telangiectasia

PART II von Willebrand Disease

SECTION I Management during Procedures

CASE STUDY 28 Type 1 von Willebrand Disease and Tonsillectomy

CASE STUDY 29 von Willebrand Disease and Dental Surgery

CASE STUDY 30 von Willebrand Disease and Gastrointestinal Surgery

Gynecologic and Obstetric Considerations: von Willebrand Disease and Obstetric/Gynecologic Procedures

SECTION II Rare Forms of von Willebrand Disease

CASE STUDY 31 Type 2A von Willebrand Disease and Recurrent Gastrointestinal Bleeding

CASE STUDY 32 Type 2B von Willebrand Disease and Thoracic Surgery

CASE STUDY 33 von Willebrand Disease 2N

PART III Other Bleeding Disorders

CASE STUDY 34 Prothrombin Deficiency

CASE STUDY 35 Factor V Deficiency

CASE STUDY 36 Factor VII Deficiency

CASE STUDY 37 Factor X Deficiency

CASE STUDY 38 Factor XI Deficiency

CASE STUDY 39 Factor XIII Deficiency

CASE STUDY 40 Combined Factor V and Factor VIII Deficiency

CASE STUDY 41 Glanzmann Thrombaesthenia

CASE STUDY 42 Gardner–Diamond Syndrome and von Willebrand Disease

CASE STUDY 43 Qualitative Platelet Disorder

PART IV Acquired Bleeding Disorders

CASE STUDY 44 Acquired FVIII Inhibitor and B Cell Neoplasm

CASE STUDY 45 FVIII Inhibitor and Lupus Inhibitor

CASE STUDY 46 Acquired von Willebrand Disease

CASE STUDY 47 A Woman with Bleeding Gums

CASE STUDY 48 Bleeding after Cardiac Surgery

CASE STUDY 49 Bleeding in a Dialysis Patient

CASE STUDY 50 A Woman with Anemia and Hematuria

CASE STUDY 51 Scalp Bleeding in an Older Gentleman

CASE STUDY 52 Hyperfibrinolysis

PART V Thrombotic Disorders

CASE STUDY 53 Heparin-Induced Thrombocytopenia with Thrombosis

CASE STUDY 54 Heparin Skin Necrosis

CASE STUDY 55 Warfarin Skin Necrosis

CASE STUDY 56 Thoracic Outlet Syndrome

CASE STUDY 57 Antithrombin Deficiency

CASE STUDY 58 May–Thurner Syndrome

CASE STUDY 59 Thrombosis in a Liver Transplant Patient

CASE STUDY 60 Combined Thrombophilia

Index

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Library of Congress Cataloging-in-Publication Data

Hemophilia and hemostasis : a case-based approach to management / edited by Alice D. Ma,Harold R. Roberts, Miguel A. Escobar. -- 2nd ed.p. ; cm.Rev. ed. of: Haemophilia and haemostasis. c2007.Includes bibliographical references and index.

ISBN 978-0-470-65976-2 (hardback : alk. paper)I. Ma, Alice. II. Roberts, H. R. (Harold Ross) III. Escobar, Miguel A. IV. Haemophilia and haemostasis.[DNLM: 1. Blood Coagulation Disorders, Inherited--therapy--Case Reports. 2. Thrombosis--therapy--Case Reports. WH 322]616.1'572--dc23

2012017384

A catalogue record for this book is available from the British Library.

Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.

Contributors

Anas Alrwas, MDResident, Internal MedicineUniversity of Texas Health Science Center at HoustonHouston, TX, USATyler Buckner, MDHematology FellowPediatric Hematology/OncologyAdult HematologyUniversity of North Carolina School of MedicineChapel Hill, NC, USABenjamin Carcamo, MDClinical Assistant ProfessorPediatric Hematology OncologyProvidence Memorial HospitalTexas Tech University, School of MedicineEl Paso, TX, USAMiguel A. Escobar, MDAssociate Professor of Medicine and PediatricsDivision of HematologyUniversity of Texas Health Science Center at HoustonDirector, Gulf States Hemophilia and Thrombophilia CenterHouston, TX, USAMatthew Foster, MDAssistant Professor of MedicineDivision of Hematology/OncologyUniversity of North Carolina School of MedicineChapel Hill, NC, USARaj Sundar Kasthuri, MDAssistant Professor of MedicineDivision of Hematology/OncologyUniversity of North Carolina School of MedicineChapel Hill, NC, USAKristy Lee, MS, CGCClinical Assistant ProfessorDepartment of GeneticsUniversity of North Carolina School of MedicineChapel Hill, NC, USAAlice D. Ma, MDAssociate Professor of MedicineDivision of Hematology/OncologyUniversity of North Carolina School of MedicineChapel Hill, NC, USAMarshall Mazepa, MDSenior FellowDivision of Hematology/OncologyUniversity of North Carolina School of MedicineChapel Hill, NC, USATrinh T. Nguyen, DOAssistant Professor of PediatricsDivision of HematologyUniversity of Texas Health Science Center at HoustonGulf States Hemophilia and Thrombophilia CenterHouston, TX, USANidra Rodriguez, MDAssistant Professor of PediatricsDivision of HematologyThe University of Texas Health Science Center at HoustonMD Anderson Cancer CenterGulf States Hemophilia & Thrombophilia CenterHouston, TX, USAE. Carlos Rodriguez-Merchan, MD, PhDConsultant Orthopaedic Surgeon and Associate Professor of OrthopaedicsLa Paz University HospitalUniversidad AutonomaMadrid, SpainJenny M. Splawn, PharmDProvidence Memorial HospitalEl Paso, TX, USATzu-Fei Wang, MDFellowDivisions of Hematology and OncologyWashington University School of MedicineSaint Louis, MO, USA

Foreword

I am delighted to respond to the invitation to provide a brief introduction to the second edition of Hemophilia and Hemostasis: A Case-Based Approach to Management. The popularity of this text stems from its unique case-based approach. Drs Roberts, Ma, and Escobar are renowned and frequently consulted experts in the management of patients with bleeding disorders. Although the hemophilias and other inherited bleeding disorders have been the focus of a comparatively large body of literature, there are remarkably few randomized-controlled clinical trials on which to base firm evidence-based recommendations. This fact was most recently brought home to me as a member of the team charged with revising the World Federation of Hemophilia’s Treatment Guidelines; our goal was to provide appropriately graded recommendations of the literature and generally accepted practices for the practicing clinician. Unfortunately, the paucity of high-quality level 1 evidence does not obviate the need to make clinical decisions on a daily basis when caring for patients with bleeding disorders. The authors address these management dilemmas in a comprehensive series of “mini-chapters” that provide an easy reference format for the reader. In this day and age of electronic fingertip access to state-of-the-art reviews on PubMed, it is sometimes said that textbooks are obsolete before they are even published. While there may be some truth to this viewpoint in the case of standard texts, no amount of electronic searching can provide the ready access to the august consensus opinions of these seasoned experts, who have “been down that same road” before. As such, this book is a must for every hematologist or nurse who is charged with taking care of patients with bleeding disorders.

Nigel S. Key MB ChB FRCPHarold R. Roberts Distinguished Professor of Medicineand Pathology and Laboratory MedicineChief, Section of HematologyDirector, UNC Hemophilia and Thrombosis CenterChapel Hill, NC

PART I

Hemophilia A and Hemophilia B

SECTION I

General Overview

INTRODUCTION 1

The Hemophilic Ankle: An Update

E. Carlos Rodriguez-Merchan

La Paz University Hospital and Universidad Autonoma, Madrid, Spain

Q  What is the latest information regarding the treatment of hemophilic arthropathy in the ankle?

It is well known that the ankles in hemophilic patients tend to bleed, beginning at an early age of 2–5 years. The synovium is only able to reabsorb a small amount of intra-articular blood; if the amount of blood is excessive, the synovium will hypertrophy as a compensating mechanism, so that eventually the affected joint will show an increase in size of the synovium, leading to hypertrophic chronic hemophilic synovitis. The hypertrophic synovium is very richly vascularized, so that small injuries will easily make the joint rebleed. The final result will be the vicious cycle of hemarthrosis–synovitis–hemarthrosis, which eventually will result in hemophilic arthropathy ().