Inhibitors in Patients with Haemophilia (eBook)

E. C. Rodriquez-Merchan is the editor of Inhibitors in Patients with Haemophilia, published by Wiley. Christine A. Lee is the editor of Inhibitors in Patients with Haemophilia, published by Wiley.

Inhibitors in Patients with Haemophilia 5
Contents 7
Contributors 9
Preface 12
Part 1: Haematology 13
1 Natural history of inhibitors in severe haemophilia A and B: incidence and prevalence 15
2 Characterization of inhibitors in congenital haemophilia 21
3 Incidence and prevalence of inhibitors and type of blood product in haemophilia A 26
4 Genetic basis of inhibitor development in severe haemophilia A and B 33
Part 2: Management of treatment 39
5 Methods: plasmapheresis and protein A immunoadsorption 41
6 Venous access in children with inhibitors 48
Part 3: Immune tolerance 55
7 Immune tolerance: high-dose regimen 57
8 Immune tolerance: low-dose regimen 61
9 Immune tolerance and choice of concentrates 67
10 Immune tolerance: The North American Immune Tolerance Registry 71
Part 4: Medical management of bleeding episodes 79
11 The treatment of bleeding episodes in children 81
12 General medical management of bleeding episodes: haemarthroses, muscle haematomas, mucocutaneous bleeding and haematuria 86
13 Life- and limb-threatening episodes and intracranial bleeds 90
Part 5: Inhibitors in haemophilia B, inhibitors in mild and moderate haemophilia A and acquired inhibitors to FVIII 97
14 Factor IX inhibitors and anaphylaxis 99
15 Inhibitors in mild and moderate haemophilia A 104
16 Acquired inhibitors to factor VIII 110
Part 6: Musculoskeletal issues 125
17 Pathogenesis of musculoskeletal complications of haemophilia 127
18 Orthopaedic management of haemarthroses 132
19 Chemical synoviorthesis 138
20 Radiosynoviorthesis in patients with haemophilia and inhibitors 141
21 Musculoskeletal magnetic resonance imaging 144
22 Treatment of iliopsoas haematomas and compartment syndromes in patients with haemophilia who have a circulating antibody 151
23 A rational approach to the treatment of haemophilic blood cyst (pseudotumour) in patients with inhibitors 154
24 Haemophilia patients with inhibitors: a rheumatologist's point of view 158
25 Rehabilitation of patients with haemophilia and inhibitors 161
26 Physiotherapy in the management of patients with inhibitors 172
27 Elective orthopaedic surgery in haemophilia patients with high-responding inhibitors 181
Part 7: General surgery 189
28 General and emergency surgery in patients with high-responding inhibitors 191
29 Dental extraction in patients with haemophilia and inhibitors 195
Part 8: Psychosocial issues 197
30 Psychosocial impact of inhibitors on haemophilia patients' quality of life 199
Part 9: General strategy for management of inhibitor patients 205
31 General strategy for management of inhibitor patients (summary) 207
32 Immunoadsorption with anti-immunoglobulin antibodies using Ig-TheraSorb columns 211
33 Standard, high and mega bolus doses of rVIIa or recombinant VIIa: comparison of three different treatment regimens 218
Index 223