The only up-to-date definitive reference source on hemophiliaThis book is an invaluable resource that provides an overview of all aspects of the care of patients with haemophilia.Covering how to assess both bleeding children and adults, Haemophilia A and B, molecular basis of the disease, the role of factors in coagulation, epidemiology, pharmacokinetics, and treatment of inhibitors. There will also be a section on musculoskeletal aspects of haemophilia as well as newer developments such as gene therapy and rare bleeding disorders.Textbook of Hemophilia is ideal for: Trainees and residents in hematology Hematologists in practice Specialists working in thrombosis and hemostasis as well as transfusion medicineWhy Buy This Book?The only up-to-date definitive reference source on hemophilia Essential for all those managing hemophilia patients Detailed guidance on assessment, diagnosis, management and treatment Advice for everyday clinical questions Edited by three of the world's leading experts on hemophilia

Professor Christine A. Lee, Consultant in Haematology, Royal Free Hospital, London

Dr Keith Hoots, Gulf State Hemophilia Center, University of Texas Health Sciences Center, USA

Dr Eric Berntop, Malmo University Hospital, Sweden

Contributors

vii

Foreword

xi

Part I Introduction

Overview of hemostasis

1

(4)

Kenneth G. Mann

Kathleen Brummel Ziedins

Cellular processing of factors VIII and IX

5

(8)

Randal J. Kaufman

Work-up of a bleeding adult

13

(6)

Barry White

Clodagh Ryan

Part II Hemophilia A

Molecular basis of hemophilia A

19

(8)

Geoffrey Kemball-Cook

Edward Tuddenham

Hemophilia A: role of factor VIII in coagulation

27

(7)

Evgueni L. Saenko

Natalya M. Ananyeva

Natural history of inhibitor development in children with severe hemophilia A treated with factor VIII products

34

(5)

Jeanne M. Lusher

Prophylaxis

39

(7)

H. Marijke van den Berg

Kathelijn Fischer

Continuous infusion of coagulation products in hemophilia

46

(7)

Uri Martinowitz

Angelika Batorova

Part III Inhibitors to factor VIII

Inhibitors to factor VIII--immunology

53

(6)

Jean-Marie R. Saint-Remy

Marc G. Jacquemin

Inhibitors to factor VIII--molecular basis

59

(5)

Johannes Oldenburg

Edward Tuddenham

Inhibitors to factor VIII--epidemiology and treatment

64

(7)

Donna DiMichele

Inhibitors to factor VIII--mild and moderate hemophilia

71

(3)

Kathelijne Peerlinck

Marc G. Jacquemin

Inhibitors to factor VIII/IX: treatment of inhibitors--immune tolerance induction

74

(6)

Charles R.M. Hay

Inhibitors of factor VIII: treatment of acute bleeds

80

(6)

Claude Negrier

Part IV Acquired hemophilia

Acquired inhibitors to factor VIII

86

(5)

Craig M. Kessler

Ekatherine Asatiani

Part V Hemophilia B

Hemophilia B--molecular basis

91

(6)

Peter M. Green

Inhibitors in hemophilia B

97

(4)

Indira Warrier

Treatment of inhibitors in hemophilia B

101

(5)

Simon A. Brown

Part VI Pharmacokinetics of factors VIII and IX

Pharmacokinetics

106

(6)

Sven Bjorkman

Erik E. Berntorp

Part VII Pediatrics

Work-up of a bleeding child

112

(8)

Victor S. Blanchette

Walter H.A. Kahr

Care of the child with hemophilia

120

(5)

Rolf Ljung

The neonate with hemophilia

125

(6)

Angela Thomas

Elizabeth Chalmers

Part VIII Products used to treat hemophilia

Products used to treat hemophilia: evolution of treatment for hemophilia A and B

131

(5)

Inge Scharrer

Thomas Becker

Products used to treat hemophilia: recombinant products

136

(6)

Akira Yoshioka

Products used to treat hemophilia: plasma-derived coagulation factor concentrates

142

(5)

Paul L.F. Giangrande

Products used to treat hemophilia: recombinant factor VIIa

147

(6)

Ulla Hedner

Products used to treat hemophilia: dosing

153

(5)

Miguel A. Escobar

Products used to treat hemophilia: regulation

158

(6)

Albert Farrugia

Part IX Musculoskeletal

Joint replacement

164

(5)

Nicholas J. Goddard

Synoviorthesis in hemophilia

169

(5)

E. Carlos Rodriguez-Merchan

Pseudotumors in patients with hemophilia

174

(3)

Michael Heim

Uri Martinowitz

Radiology

177

(5)

Holger Pettersson

Magnetic resonance imaging/joint outcome assessment

182

(11)

Marilyn J. Manco-Johnson

Ray F. Kilcoyne

Physiotherapy in the management of hemophilia

193

(7)

Karen Beeton

Jane Tuffley

Part X Transfusion-transmitted disease

Transfusion transmitted disease: history of epidemics (focus on HIV)

200

(7)

W. Keith Hoots

Transfusion transmitted disease: hepatitis C virus infection and liver transplantation

207

(7)

Margaret V. Ragni

Part XI Gene therapy

Gene therapy: introduction and overview

214

(6)

Gilbert C. White II

R. Jude Samulski

Gene therapy for hemophilia B

220

(6)

Katherine P. Ponder

Gene therapy for hemophilia A

226

(3)

Gilbert C. White II

Paul E. Monahan

Gene therapy: molecular engineering of factor VIII and factor IX

229

(6)

David Lillicrap

Part XII Laboratory

Laboratory assays in hemophilia

235

(7)

Jørgen Ingerslev

Standardization of assays

242

(7)

Trevor W. Barrowcliffe

Part XIII Obstetrics and gynecology

Obstetrics and gynecology: hemophilia

249

(8)

Rezan Kadir

Christine A. Lee

Part XIV von Willebrand disease

von Willebrand disease: molecular aspects

257

(8)

Ulrich Budde

Reinhard Schneppenheim

von Willebrand disease: epidemiology

265

(7)

Francesco Rodeghiero

Giancarlo Castaman

von Willebrand disease: biological diagnosis

272

(7)

Edith Fressinaud

Dominique Meyer

Classification and clinical aspects of von Willebrand disease

279

(6)

Augusto B. Federici

Part XV Treatment of von Willebrand disease

Treatment of von Willebrand disease: desmopressin

285

(4)

Pier M. Mannucci

Treatment of von Willebrand disease: therapeutic concentrates

289

(7)

Peter Collins

Women and von Willebrand disease

296

(6)

Peter A. Kouides

Part XVI Rare bleeding disorders

Factor II

302

(4)

Jan Astermark

Factor V and combined factor V and VIII deficiencies

306

(5)

Flora Peyvandi

Marta Spreafico

Congenital factor VII deficiency

311

(4)

Guglielmo Mariani

Alberto Dolce

Factor X and factor X deficiency

315

(6)

David J. Perry

Factor XI deficiency

321

(7)

Uri Seligsohn

Factor XIII

328

(4)

Hans H. Brackmann

Vytautas Ivaskevicius

Fibrinogen

332

(6)

Michael Laffan

Miscellaneous rare bleeding disorders

338

(7)

Amy Shapiro

Rekha Parameswaran

Part XVII Quality of life

Quality of life in hemophilia

345

(6)

Sylvia v. Mackensen

Alessandro Gringeri

Part XVIII Economics

The economics of hemophilia treatments

351

(8)

Alec H. Miners

Part XVIV Comprehensive care and delivery of care

Comprehensive care and delivery of care: the developed world

359

(7)

Christopher A. Ludlam

Comprehensive care and delivery of care: the developing world

366

(5)

Alok Srivastava

Auro Viswabandya

Comprehensive care and delivery of care: the global perspective

371

(8)

Bruce L. Evatt

Claudia Black

Index

379

What is included with this book?

The New copy of this book will include any supplemental materials advertised. Please check the title of the book to determine if it should include any access cards, study guides, lab manuals, CDs, etc.

The Used, Rental and eBook copies of this book are not guaranteed to include any supplemental materials. Typically, only the book itself is included. This is true even if the title states it includes any access cards, study guides, lab manuals, CDs, etc.