Textbook of Hemophilia

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

• ISBN13: 9781444318555
• ISBN10: 1444318551
• Edition: 2nd
• Format: eBook
• Copyright: 2010-08-13
• Publisher: Wiley-Blackwell

Summary

The Textbook of Hemophilia has become a definitive resource for all those managing hemophilia patients. It covers all the common and rare bleeding disorders, both in terms of clinical management as well as the genetic, laboratory, financial and psychological aspects.

This second edition covers all the latest developments in the field of hemophilia, with new chapters on:

• the genetic and molecular basis of inherited blood disorders
• how to manage adolescent and older patients
• emergency medicine and inherited blood disorders
• national hemophilia databases

Drawing on the vast experience of the authors, the aim of this textbook remains the same - to improve the care of patients suffering from hemophilia. The book is full of detailed guidance and advice on everyday clinical questions making it invaluable to all trainee and practicing hematologists.

Author Biography

Christine A. Lee MA, MD, DSc, FRCP, FRCPath
Emeritus Professor of Haemophilia, University of London;
Honorary Consultant Haematologist, Oxford Haemophilia & Thrombosis Centre, Oxford, UK

Erik E. Berntorp MD, PhD
Professor of Haemophilia, 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

Table of Contents

List of Contributors.

Historical introduction (Christine A. Lee).

Part I: Introduction.

1 Overview of Hemostasis (Kathleen Brummel Ziedins and Kenneth G. Mann).

2 Cellular processing of Factor VIII and Factor IX (Michael U. Callaghan and Randal J. Kaufman).

Part II: Hemophilia A.

3 Molecular Basis of Hemophilia A (Geoffrey Kemball-Cook and Keith Gomez).

4 Phenotypic/genotypic relationship (Kathelijn Fischer and H. Marijke van den Berg).

5 Prophylaxis (Kathelijn Fischer and H. Marijke van den Berg).

6 Continuous Infusion of Coagulation Products in Hemophilia (Angelika Batorova and Uri Martinowitz).

Part III: Inhibitors to Factor VIII.

7 Inhibitors to Factor VIII - Immunology (Jean-Marie R. Saint-Remy and Marc G. Jacquemin).

8 Genetic and environmental risk factors for FVIII inhibitor development (Jan Astermark).

9 Inhibitors to Factor VIII - Mild and Moderate Hemophilia (Kathelijne Peerlinck and Marc Jacquemin).

10 Inhibitors to Factor VIII/IX: Immune Tolerance (Donna DiMichele).

11 Inhibitors to factor VIII: treatment of acute bleeds (Claude Negrier).

Part IV: Acquired Hemophilia.

12 Acquired Inhibitors to Factor VIII (Suman L. Sood and Craig M. Kessler).

Part V: Hemophilia B.

13 Hemophilia B - Molecular Basis (Keith Gomez).

14 Factor IX Inhibitors in Hemophilia B (Jeanne M. Lusher and Meera Chitlur).

15 Treatment of Inhibitors in Hemophilia B (Anand Tandra and Amy D. Shapiro).

Part VI: Pharmacokinetics of Factors VIII & IX.

16 Pharmacokinetics (Sven Björkman and Erik E. Berntorp).

Part VII: Hemophilia - birth to old age.

17 The Neonate with Hemophilia (Angela E. Thomas and Elizabeth Chalmers).

18 Work-up of a Bleeding Child (Manuel D. Carcao and Victor S. Blanchette).

19 Care of the Child with Hemophilia (Rolf Ljung).

20 Adolescence (Martti A Siimes).

21 Old age medicine and hemophilia (Evelien P. Mauser-Bunschoten and R.E.G. Schutgens).

Part VIII: Products used to Treat Hemophilia.

22 Products Used to Treat Hemophilia: Recombinant Products (Akira Yoshioka).

23 Products Used to Treat Hemophilia: Plasma-Derived Coagulation Factor Concentrates (Paul Giangrande).

24 Products Used to Treat Hemophilia: Recombinant Factor VIIa (Ulla Hedner).

25 Products Used to Treat Hemophilia: Dosing (Miguel A. Escobar).

26 Products Used to Treat Hemophilia: Regulation (Albert Farrugia).

Part IX: Musculoskeletal.

27 Joint Replacement (Nicholas Goddard).

28 Synovioarthesis in Hemophilia (E. Carlos Rodriguez-Merchan).

29 Pseudotumours in patients with Hemophilia (Michael Heim and Uri Martinowitz).

30 Imaging modalities for assessment of hemophilic arthropathy (Andrea S. Doria and Bjorn Lundin).

31  Physiotherapy in the Management of Hemophilia (Karen Beeton and Jane Padkin).

32 Clinimetric Instruments in Haemophilia (Pradeep M. Poonnoose and Frank van Genderen).

Part X: Transfusion-Transmitted Disease.

33 Hepatitis C Virus Infection and Liver Transplantation (Michael Makris and Geoffrey Dusheiko).

34 Emerging Infections (Thomas R Kreil).

Part XI: Gene Therapy.

35 Hemophilia Gene Therapy: An overview (David Lillicrap).

36 Gene Therapy Trials (Kathy High).

37 Gene Therapy: Molecular Engineering of Factor VIII and Factor IX (Steven W. Pipe).

Part XII: Laboratory.

38 Laboratory Assays in Hemophilia (Jorgen Ingerslev).

39 Standardization of Assays (Sanj Raut and Trevor W. Barrowcliffe).

40 Global laboratory assays in Haemophilia (Benny Sørensen and Guy Young).

Part XIII: Obstetrics & Gynaecology.

41 Obstetrics and Gynecology: Hemophilia (Rezan A Kadir and Christine A. Lee).

Part XIV: Von Willebrand Disease.

42 von Willebrand Disease: Molecular Aspects (Anne Goodeve).

43 von Willebrand Disease: Epidemiology (Francesco Rodeghiero and Giancarlo Castaman).

44 von Willebrand Disease: Biological Diagnosis (Bob Montgomery and Veronica Flood).

45 Classification and Clinical Aspects of von Willebrand Disease (Augusto Federici).

Part XV: Treatment of Von Willebrand Disease.

46 Treatment of von Willebrand Disease: Desmopressin (Pier Mannuccio Mannucci).

47 Treatment of von Willebrand Disease: Therapeutic Concentrates (Peter Collins).

48 Women & Von Willebrand Disease (Peter Kouides).

Part XVI: Rare Bleeding Disorders.

49 Factor II (Jan Astermark).

50 Factor V and combined Factor V and VIII Deficiencies (Flora Peyvandi and Marta Spreafico).

51 Congenital Factor VII Deficiency (Guglielmo Mariani and Alberto Dolce).

52 Factor X  Deficiency (David J Perry).

53 Factor XI Deficiency (Uri Seligsohn and Paula Bolton Maggs).

54  Factor XIII Deficiency (Diane Nugent and Loan Hsieh).

55 Fibrinogen Deficiency (Michael Laffan).

56 Miscellaneous Rare Bleeding Disorders (Anjali A. Sharathkumar and Amy D. Shapiro).

Part XVII: Emergency medicine.

57 Emergency Medicine and Inherited Blood Disorders (W. Keith Hoots).

Part XVIII: Quality of Life.

58 Quality of Life in Hemophilia (Sylvia v. Mackensen and Alessandro Gringeri).

Part XVIII: Economics.

59 The Economics of Hemophilia Treatments (Katarina Steen Carlsson and Erik Berntorp).

Part XVIV: Comprehensive Care and Delivery of Care.

60 National Haemophilia Databases (Charles R.M. Hay).

61 Comprehensive Care and the Delivery of Care: The Developed World (Christopher A Ludlam).

62 Comprehensive care and delivery of care in haemophilia: The Developing World (Alok Srivastava and Auro Viswabandya).

63 Comprehensive Care and Delivery of Care: The Global Perspective (Bruce L. Evatt and Alison Street).

Index.