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Textbook of Hemophilia, 3rd Edition

Textbook of Hemophilia, 3rd Edition

Christine A. Lee, Erik E. Berntorp, W. Keith Hoots

ISBN: 978-1-118-39828-9

Apr 2014, Wiley-Blackwell

560 pages



Textbook of Hemophilia, 3rd edition

Edited by
Christine A. Lee, MA, MD, DSc, FRCP, FRCPath, FRCOG
Emeritus Professor of Haemophilia, University of London, London, UK

Erik E. Berntorp, MD, PhD
Professor of Coagulation Medicine, Lund University
Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden

W. Keith Hoots, MD
Director, Division of Blood Diseases and Resources, National Heart, Lung and Blood Institute
National Institutes of Health, Bethesda, MD;
Professor of Pediatrics and Internal Medicine, University of Texas Medical School at Houston, Houston, TX, USA

Without doubt, Textbook of Hemophilia, 3rd edition is the definitive reference source on all aspects of haemophilia including diagnosis, management and treatment. Edited by three, world-renowned experts on haemophilia, this completely revised resource features chapters written by over 60 international contributors with international expertise in caring for haemophilia patients.

Textbook of Hemophilia, 3rd edition

  • Features eight new chapters, covering individualised dosing, vCJD and haemophilia, new drugs in the pipeline, and surgery in inhibitor patients
  • Presents new developments, such as gene therapy
  • Highlights controversial issues and provides advice for everyday clinical questions
  • Represents essential reading for all healthcare professionals involved in the care of those with haemophilia

 Titles of related interest

 Hemophilia and Hemostasis: A Case-Based Approach to Management, 2nd Edition

Ma, ISBN: 9780470659762

Current and Future Issues in Hemophilia Care

Rodriguez-Merchan, ISBN: 9780470670576

Contributors, ix

Historical introduction, xv
Christine A. Lee

PART I: Introduction

1 Overview of hemostasis, 3
Kathleen Brummel Ziedins and Kenneth G. Mann

2 Cellular processing of factor VIII and factor IX, 9
Michael U. Callaghan and Randal J. Kaufman

PART II: Hemophilia A

3 Molecular basis of hemophilia A, 23
Geoffrey Kemball-Cook and Keith Gomez

4 Prophylaxis, 33
Kathelijn Fischer and H. Marijke van den Berg

PART III: Inhibitors to factor VIII

5 Inhibitors to factor VIII: immunology, 43
Jean-Marie R. Saint-Remy and Marc G. Jacquemin

6 Genetic and environmental risk factors for factor VIII inhibitor development, 48
Jan Astermark

7 Epidemiology of inhibitors in hemophilia, 53
Alfonso Iorio

8 Inhibitors to factor VIII: mild and moderate hemophilia, 59
Kathelijne Peerlinck and Marc Jacquemin

9 Inhibitors to factor VIII/IX: immune tolerance, 64
Donna M. DiMichele

10 Prophylaxis in inhibitor patients, 72
Alessandro Gringeri

11 Inhibitors to factor VIII: treatment of acute bleeds, 78
Claude Negrier

PART IV: Acquired hemophilia

12 Acquired inhibitors to factor VIII, 87
Craig M. Kessler

PART V: Hemophilia B

13 Hemophilia B: molecular basis, 97
Keith Gomez and Pratima Chowdary

14 Factor IX inhibitors in hemophilia B, 103
Meera B. Chitlur and Jeanne M. Lusher

15 Treatment of inhibitors in hemophilia B, 107
Anand Tandra and Amy D. Shapiro

PART VI: Pharmacokinetics of factors VIII and IX

16 Pharmacokinetics, 117
Sven Björkman

17 Individualized dosing, 123
Peter W. Collins

PART VII: Hemophilia: birth to old age

18 Neonate with hemophilia, 131
Angela E. Thomas and Elizabeth A. Chalmers

19 Work-up of a bleeding child, 138
Manuel D. Carcao and Victor S. Blanchette

20 Care of the child with hemophilia, 145
Rolf C.R. Ljung

21 Hemophilia in adolescence, 150
Pia Petrini

22 Old age medicine and hemophilia, 154
Evelien P. Mauser-Bunschoten and Roger E.G. Schutgens

PART VIII: Products used to treat hemophilia

23 Products used to treat hemophilia: recombinant products, 165
Midori Shima and Akira Yoshioka

24 Products used to treat hemophilia: plasma-derived coagulation factor concentrates, 174
Paul L.F. Giangrande

25 Products used to treat hemophilia: dosing, 180
Miguel A. Escobar

26 Products used to treat hemophilia: regulation, 185
Albert Farrugia

27 New drugs in the pipeline: from concept to clinic, 192
Leonard A. Valentino

PART IX: Surgical management

28 General surgical management of patients with hemophilia, 199
Cindy Leissinger and Rebecca Kruse-Jarres

29 Continuous infusion of coagulation products in hemophilia, 204
Angelika Batorova and Uri Martinowitz

30 Surgery in inhibitor patients, 213
Pål Andrè Holme

PART X: Musculoskeletal

31 Joint replacement in patients with hemophilia, 221
Nicholas Goddard

32 Medical synovectomy (synoviorthesis) in hemophilia: radiosynovectomy and chemical synovectomy, 228
E. Carlos Rodriguez-Merchan

33 Pseudotumors in patients with hemophilia, 233
Michael Heim and Uri Martinowitz

34 Imaging modalities for assessment of hemophilic arthropathy, 237
Andrea S. Doria and Björn Lundin

35 Physiotherapy in the management of hemophilia, 247
Sébastien Lobet and David Stephensen

36 Outcome assessment in hemophilia, 253
Pradeep M. Poonnoose and Alok Srivastava

PART XI: Transfusion-transmitted disease

37 Viral hepatitis and hemophilia, 265
Michael Makris and Geoffrey Dusheiko

38 Transfusion-transmitted disease: emerging infections, 272
Thomas R. Kreil

39 vCJD and hemophilia, 277
Carolyn M. Millar

PART XII: Gene therapy

40 Hemophilia gene therapy: an overview, 285
David Lillicrap

41 Gene therapy trials in hemophilia A and B, 291
Katherine A. High

42 Gene therapy: molecular engineering of factor VIII and factor IX, 298
Sundar R. Selvaraj and Steven W. Pipe

PART XIII: Laboratory

43 Laboratory and quality control of assays, 311
Steve Kitchen

44 Standardization of assays in hemophilia, 318
Sanj Raut and Trevor W. Barrowcliffe

45 Global laboratory assays in hemophilia, 328
Benny Sørensen and Guy Young

PART XIV: Women and bleeding disorders

46 Obstetrics and gynecology: hemophilia, 337
Rezan A. Kadir and Christine A. Lee

47 Women and von Willebrand disease, 345
Peter A. Kouides

PART XV: von Willebrand disease

48 von Willebrand disease: molecular aspects, 355
Daniel Hampshire and Anne Goodeve

49 von Willebrand disease: epidemiology, 362
Francesco Rodeghiero and Giancarlo Castaman

50 von Willebrand disease: biological diagnosis, 370
Veronica H. Flood and Robert R. Montgomery

51 Classification and clinical aspects of von Willebrand disease, 377
Augusto B. Federici

52 Treatment of von Willebrand disease: desmopressin, 386
Pier M. Mannucci

53 Treatment of von Willebrand disease: therapeutic concentrates, 390
Erik E. Berntorp

PART XVI: Rare bleeding disorders

54 Factor II, 399
Jan Astermark

55 Factor V and combined factor V and VIII deficiencies, 403
Flora Peyvandi and Marzia Menegatti

56 Congenital factor VII deficiency, 413
Angelika Batorova

57 Factor X and factor X deficiency, 421
David J. Perry

58 Factor XI deficiency, 428
Paula H.B. Bolton-Maggs and Uri Seligsohn

59 Factor XIII deficiency, 436
Diane Nugent and Loan Hsieh

60 Fibrinogen deficiency, 445
Michael Laffan

61 Miscellaneous rare bleeding disorders, 452
Frederico Xavier and Amy D. Shapiro

PART XVII: Emergency medicine

62 Emergency management of hemophilia, 463
W. Keith Hoots

PART XVIII: Evaluation of hemophilia

63 Clinical trials and other methodologies, 473
Sharyne M. Donfield and Alice E. Lail

64 Quality of life in hemophilia, 478
Sylvia von Mackensen and Alessandro Gringeri

65 The economics of hemophilia treatment, 489
Katarina Steen Carlsson and Erik E. Berntorp

PART XIX: Comprehensive care and delivery of care

66 Hemophilia databases, 497
Charles R.M. Hay

67 Comprehensive care and delivery of care: the developed world, 502
Christopher A. Ludlam and Cedric R.J.R. Hermans

68 Comprehensive care and delivery of care in hemophilia: the developing world, 508
Alok Srivastava and Auro Viswabandya

69 Comprehensive care and delivery of care: the global perspective, 515
Mark W. Skinner and Alison M. Street

Index, 523

Color plate section can be found facing page 202