Haemophilia and Haemostasis: A Case-based Approach to Management PDF
Preview Haemophilia and Haemostasis: A Case-based Approach to Management
Haemophilia and Haemostasis Special thanks to Novo Nordisk Haemophilia and Haemostasis: A Case-based Approach to Management Compiled from the Haemostasis/Haemophilia Forum EDITED BY Harold Ross Roberts, MD Division of Hematology/Oncology University of North Carolina Medical School Chapel Hill, NC, USA ASSOCIATE EDITORS Daniel Carrizosa, MD Division of Hematology/Oncology University of North Carolina Medical School Chapel Hill, NC, USA Alice Ma, MD Division of Hematology/Oncology University of North Carolina Medical School Chapel Hill, NC, USA © 2007 by Blackwell Publishing Blackwell Publishing, Inc., 350 Main Street, Malden, MA 02148-5020, USA Blackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ, UK Blackwell Publishing Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia The right of the Author to be identifi ed as the Author of this Work has been asserted in accordance with the Copyright, Designs and Patents Act 1988. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. First published 1 2007 Library of Congress Cataloging-in-Publication Data Haemophilia and haemostasis : a case-based approach to management / compiled from the Haemostasis/haemophilia forum ; edited by Haro ld Ross Roberts ; associate editors, Daniel Carrizosa, Alice Ma. p.; cm. Includes bibliographic references and index. ISBN 978-1-4051-6716-1 (alk. paper) 1. Hemophilia. 2. Hemostasis. 3. Blood coagulation disorders. I. Roberts, H. R. (Harold Ross) II. Carrizosa, Daniel. III. Ma, Alice. IV. Haemostasis forum. [DNLM: 1. Blood Coagulation Disorders, Inherited—therapy—Collected Works. 2. Thrombosis—therapy—Collected Works.—WH 322 H134 2007] RC642.H325 2007 616.1(cid:1)572—dc22 2007010316 ISBN: 978-1-4051-6716-1 A catalogue record for this title is available from the British Library Set in 10/13 Meridien by Charon Tec Ltd (A Macmillan Company), Chennai, India Printed and bound in the United Kingdom by TJ International Ltd, Padstow Commissioning Editor: Maria Khan Editorial Assistant: Jennifer Seward Development Editor: Elisabeth Dodds Production Controller: Debbie Wyer For further information on Blackwell Publishing, visit our website: http://www.blackwellpublishing.com The publisher’s policy is to use permanent paper from mills that operate a sustainable forestry policy, and which has been manufactured from pulp processed using acid-free and elementary chlorine-free practices. Furthermore, the publisher ensures that the text paper and cover board used have met acceptable environmental accreditation standards. Blackwell Publishing makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check that any product mentioned in this publication is used in accordance with the prescribing information prepared by the manufacturers. The author and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this book. Contents List of Haemostasis Forum Editors, ix List of Contributors, xv Preface, xix 1 Haemophilia A and Haemophilia B, 1 General Overview Haemophilia and Immune Tolerance Therapy, 3 The Haemophilic Ankle: An Update, 5 The Haemophilic Knee: An Update, 15 Issues with Complicated Diagnoses Combined Haemophilia A and B Carrier, 23 A Complex Case of Haemophilia with HIV and Hepatitis C, 25 A Case of Haemophilia B, Mild VWD, and a Factor IX Inhibitor, 29 Premature Infant with Haemophilia B, 33 Haemophilic Carriers Haemophilic Carriers and Delivery, 35 Mild Haemophilia in Women, 37 Treatment of the Pregnant Haemophiliac, 41 Anticoagulation and Haemophilia Anticoagulation for Atrial Fibrillation in a Haemophiliac, 43 Anticoagulation for a Cardiac Valve in a Haemophiliac, 47 Cardiac Catheterization in a Haemophiliac, 49 Anticoagulation for a DVT in a Haemophiliac, 51 Treatment of Haemophiliacs DDAVP for Treatment of Mild Haemophilia during Surgery, 55 Haemophilia and Haemodialysis, 57 Haemophilia and Hepatitis C Treatment, 59 Haemophilia and Physical Therapy, 63 Haemophilia and Renal Bleeds, 65 Haemophilia and Scuba Diving, 67 Haemophilia and Ventricular Septal Defect Repair, 69 Haemophilia with Hepatitis C and Recurrent Bleeding, 73 Isotretinoin in Haemophilia, 75 Laser Eye Surgery in a Haemophiliac, 77 Managing Haemophilic Pseudotumours, 79 Continuous NovoSeven: Pros and Cons, 81 i ii Contents Complications of Treatment Haemophilia B and Immune Tolerance with Anaphylaxis, 85 rFVIIa (NovoSeven), 87 Thrombosis in PCCs vs. APCCs, 91 2 Von Willebrand Disease, 93 Epidurals and VWD, 95 Anticoagulation for a Cardiac Valve in a Patient with VWD Type 1, 97 VWD Type 2A and Pregnancy, 99 VWD Type 2B and Pregnancy, 101 VWD Type 2B vs. Platelet Type, 103 Prophylaxis in VWD Type 3, 105 Platelet Type VWD, 107 3 Factor Deficiencies, 109 Combined Factor V and VIII Deficiency, 111 DVT Prophylaxis in FVII Deficiency, 113 Menorrhagia in Factor VII Deficiency, 115 Anticoagulation for Atrial Fibrillation in FX Deficiency, 119 Management of Factor X Deficiency, 121 Anticoagulation for Atrial Fibrillation in a Patient with Factor XI Deficiency, 123 Factor XI Deficiency and Surgery, 125 Prophylaxis for Patients with Factor XIII Deficiency and Intracranial Bleeding, 127 4 Rare Platelet and Coagulation Disorders, 129 Coagulation Disorders Afibrinogenaemia, 131 Dysfibrinogenaemia, 135 Hypofibrinogenaemia, 139 Gardner–Diamond Syndrome, 141 Hereditary Haemorrhagic Telangiectasia, 145 Hereditary Vitamin-K-Dependent Coagulation Factors Deficiency and Pregnancy, 151 Platelet Disorders Glanzmann’s Thrombasthaenia and Gastrointestinal Angiodysplasia, 153 Glanzmann’s Thrombasthaenia and Pregnancy, 157 rVIIa (NovoSeven®) and Wiskott–Aldrich Syndrome, 159 5 Acquired Bleeding Diatheses, 161 Acquired Haemophilia and Second Pregnancy, 163 Developing a Factor IX Inhibitor, 165 End-Stage Liver Disease and Surgery, 167 Treatment for Acute DIC, 169 6 Miscellaneous Questions, 173 Bleeding Time vs. PFA-100, 175 Cocaine and DDAVP, 177 Contents iii Hyponatraemia and DDAVP, 179 The Use of the INR, 181 Selective Serotonin Reuptake Inhibitors and Clotting Disturbances, 185 Work-up for Children with Intracranial Bleeding, 189 7 Thrombotic Disorders, 193 Are Asians Genetically Different from Westerners When it Comes to VTE?, 195 Treatment of Antithrombin Deficiency, 201 Anticardiolipin Antibody Questions, 203 Paediatric Antiphospholipid Syndrome and Recurrent Thrombosis, 205 Anticoagulation for Deep Venous Thrombosis in the Presence of an Intracranial Haemorrhage, 209 Clinical Probability Assessment for Thromboembolic Disease, 213 D-dimer in Thromboembolic Disease, 215 Progestins and Thrombosis, 217 Unknown Thrombophilia and Surgery, 221 Index, 223 Haemostasis Forum: Disclaimer The material in this book was originally from a forum created for health care professionals only. The information contained herein is for scientific communications and educational purposes only. There is no warranty or guarantee as to the timeliness, sequence, accuracy or completeness of the information presented in these pages, or any linked pages. Additionally, there are no warranties, express or implied, as to the results to be obtained from use of such information. Neither Novo Nordisk A/S nor its information providers shall be liable, regard- less of the cause or duration, for any errors, inaccuracies, omissions, or other defects in the information, nor for the untimeliness and inau- thenticity thereof or for any claims or losses by any third parties aris- ing there from occasioned thereby. In cases where drug indications are discussed the views expressed may not necessarily reflect the licensed indications and approved prescribing information for your country. You are advised to consult your country’s prescribing information and qualified health care professionals before acting on any information presented in these pages. If you have or suspect you may have any health problems you should consult your health care provider. Any change in medication or treatment should be made cautiously and only under medical supervision. viii List of Haemostasis Forum Editors Editor-in-Chief Harold Ross Roberts Division of Hematology/Oncology University of North Carolina Medical School Chapel Hill, NC, USA Associate Editors Louis M. Aledort Jørgen Ingerslev Mt Sinai School of Medicine Haemophilia Center New York, NY, USA Department of Clinical Immunology University Hospital Skejby Aarhus, Denmark Henri Bounameaux Department of Medicine Nigel S. Key University Hospital of Geneva Geneva, Switzerland Division of Hematology/Oncology University of North Carolina Medical School Charles R.M. Hay Chapel Hill, NC, USA Department of Haematology Manchester Royal Infirmary Jeanne Lusher Manchester, UK Wayne State University School of Medicine Ulla Hedner Detroit, MI, USA Professor, University of Lund Malmo, Sweden; and Peir M. Mannucci Research & Development A. Bianchi Bonomi Hemophilia & Novo Nordisk A/S, Denmark Thrombosis Center University of Milan Milan, Italy Keith Hoots Gulf States Hemophilia & Thrombosis Center Victor Marder University of Texas Houston Health School of Medicine at UCLA Houston, TX, USA Los Angeles, CA, USA ix x List of Haemostasis Forum Editors Claude Négrier Carmen Luisa Arocha-Piñango Edouard Herriot University Hospital Emeritus Researcher Lyon, France Instituto Venezolano de Investigaciones Cientifi cas Johannes Oldenburg Caracas, Venezula Institute of Experimental Haematology and Transfusion Medicine Guenter Auerswald University Clinic Bonn Comprehensive Care Center for Bonn, Germany Thrombosis and Haemostasis Klinikum Bremen-Mitte Margaret Ragni Professor Hess Children’s Hospital Bremen, Germany Division of Hematology/Oncology University of Pittsburgh Physicians Pittsburgh, PA, USA Ross Baker Thrombosis & Haemophilia Service E. Carlos Rodriguez-Merchan Royal Perth Hospital Perth, Australia Consultant Orthopaedic Surgeon Department of Orthopaedics and Hemophilia Unit Angelika Batorova La Paz University Hospital and National Haemophilia Center Associate Professor of Orthopaedics Bratislava, Slovakia Autonoma University Madrid, Spain Kenneth A. Bauer Inge Scharrer VA Boston Healthcare System; and Beth Israel Deaconess Medical Center Department of Haematology Harvard Medical School University of Mainz Needham, MA, USA Mainz, Germany Erik Berntorp Malmo University Hospital; and Editorial Board Department for Coagulation Disorders Lund University Tom Abshire Malmo, Sweden Department of Pediatric Hematology Emory University School of Medicine Rául Pérez Bianco Atlanta, GA, USA National Academy of Medicine Buenos Aires, Argentina Pantep Angchaisuksiri Divison of Hematology Paula Bolton-Maggs Department of Medicine University of Manchester Ramathibodi Hospital Manchester, UK Bangkok, Thailand Brigit Brand Jovan Antovic Haemophilia Center Karolinska University Hospital & Institute University Hospital Stockholm, Sweden Zurich, Switzerland
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