Produktbild: Immune Thrombocytopenia (ITP)

Immune Thrombocytopenia (ITP)

Aus der Reihe UNI-MED Science

44,80 €

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Beschreibung

Produktdetails

Einband

Gebundene Ausgabe

Erscheinungsdatum

13.05.2022

Abbildungen

37 Abb.

Verlag

Uni-Med

Seitenzahl

204

Maße (L/B/H)

1,2/17,5/24,5 cm

Gewicht

495 g

Auflage

3. Auflage (3rd edition)

Sprache

Englisch

ISBN

978-3-8374-1624-4

Beschreibung

Produktdetails

Einband

Gebundene Ausgabe

Erscheinungsdatum

13.05.2022

Abbildungen

37 Abb.

Verlag

Uni-Med

Seitenzahl

204

Maße (L/B/H)

1,2/17,5/24,5 cm

Gewicht

495 g

Auflage

3. Auflage (3rd edition)

Sprache

Englisch

ISBN

978-3-8374-1624-4

Herstelleradresse

UNI-MED
Alten Eichen 2
28359 Bremen
DE
info@uni-med.de

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  • Produktbild: Immune Thrombocytopenia (ITP)
  • 1.Introduction18
    2.History of ITP21
    2.1.Introduction: What is thrombocytopenic bleeding?21
    2.2.History of bleeding22
    2.3.History of the pathogenesis of ITP23
    2.4.History of management of patients with ITP23
    2.5.The therapeutic translation of IVIg to other autoimmune disorders with similar
    pathophysiology as in ITP25
    2.6.Stimulation of platelet production by megakaryocytes26
    2.7.Recent development of clinical research of ITP and new aspects of platelets themselves27
    3.Basic immunology of ITP30
    3.1.T cell and cytokine abnormalities in ITP30
    3.2.B cell abnormalities31
    3.3.Dendritic Cells (DC) in ITP31
    3.4.Environmental factors in ITP32
    3.5.Alternative modes of thrombocytopenia in ITP33
    3.6.Conclusions33
    4.Megakaryopoiesis in ITP36
    4.1.Overview of normal thrombopoiesis36
    4.2.Thrombopoiesis in ITP: evidence for accelerated platelet destruction38
    4.3.Thrombopoiesis in ITP: evidence for impaired platelet production39
    4.4.Megakaryopoiesis in ITP: evidence for direct roles of megakaryocytes40
    5.Genetics of ITP45
    5.1.Challenges in defining a genetic etiology of ITP45
    5.2.Germline single-nucleotide variant (SNV) candidate gene approach45
    5.3.Familial ITP45
    5.4.Genome-wide association studies (GWAS)46
    5.5.Future Directions47
    6.Differential diagnosis of ITP: Inherited Thrombocytopenias48
    6.1.Inherited Thrombocytopenias48
    6.1.1.Clinical and laboratory peculiarities of inherited thrombocytopenias48
    6.1.2.When to suspect inherited thrombocytopenias54
    6.2.Differential diagnosis between ITP and inherited thrombocytopenias55
    6.3.How to confirm the suspicion of inherited thrombocytopenia57
    7.Secondary ITP59
    7.1.Definition and limitation59
    7.2.Etiology59
    7.3.Diagnostic workflow60
    7.4.Genetic panel62
    7.5.Adult versus children62
    7.6.Conclusions64
    8.Standardization of definitions, terminology and outcome criteria:
    a common language in ITP67
    8.1.Criteria adopted by the IWG to harmonize terminology and definitions in ITP68
    8.2.Primary and secondary ITP and diagnostic platelet count threshold68
    8.3.Definition of the different phases and severity of the disease69
    8.4.Therapeutic goals70
    8.5.Definition of response71
    8.6.Refractory ITP: definition, therapeutic goals and response assessment73
    8.7.Clinical trial-adapted criteria for eligibility and outcome assessment75
    8.8.Application of 2009 IWG proposals in real-word and need for revision75
    8.9.Conclusions75
    9.Decision making in the management of children with ITP80
    9.1.A preference-sensitive decision80
    9.2.Shared decision making81
    9.3.Conclusion81
    10.ITP in childhood: a problem-oriented review of the management84
    10.1.Clinical presentation and diagnosis85
    10.2.Prevention from and therapy of bleeding87
    10.3.Non-intervention88
    10.4.Intervention88
    10.5.Splenectomy90
    10.6.Conclusions90
    11.ITP in adulthood93
    11.1.Treatment of adult ITP94
    11.1.1.General rules94
    11.1.2.First-line therapy (initial treatment for newly diagnosed patients)94
    11.1.3.Second-line therapy95
    11.1.4.Other second-line therapies97
    11.1.5.Patients failing multiple therapies99
    11.1.6.New second- or third- or later-line therapies in the management of adults with ITP100
    11.2.Conclusion101
    12.Management of ITP in the elderly105
    12.1.Diagnosis of ITP in the elderly105
    12.2.Treating ITP in the elderly105
    12.2.1.Intravenous immunoglobulins (IVIg)106
    12.2.2.Corticosteroids106
    12.2.3.Managing treatment failure and corticosteroid dependency106
    12.2.4.Thrombopoietin receptor agonists106
    12.2.5.Rituximab107
    12.2.6.Fostamatinib108
    12.2.7.Splenectomy108
    12.2.8.Other medications108
    12.2.9.Investigational therapies108
    13.Chronic refractory ITP110
    13.1.Practical Considerations of Refractory ITP110
    13.2.Infection-related thrombocytopenia113
    13.3.Drug-induced thrombocytopenia115
    13.4.Secondary ITP115
    13.5.Therapeutic approach116
    13.5.1.General approach to refractory ITP117
    13.5.1.1.Observation117
    13.5.1.2.Standard therapy117
    13.5.1.3.Therapy for refractory disease117
    13.6.Outcomes and sequelae121
    13.7.Summary122
    14.Immune thrombocytopenia in pregnancy127
    14.1.The differential diagnosis of thrombocytopenia during pregnancy127
    14.2.ITP in pregnancy128
    14.3.Conclusion130
    15.Drugs in ITP: mechanisms of action133
    15.1.Steroids133
    15.2.Intravenous immunoglobulin (IVIg)134
    15.3.Anti-D134
    15.4.Rituximab (anti-CD20)135
    15.5.Drugs that stimulate platelet production136
    15.6.Conclusion137
    16.Mechanism of action of high dose intravenous immunoglobulin in ITP141
    16.1.Introduction141
    16.2.Mononuclear phagocyte system (MPS) blockade142
    16.3.Anti-idiotype antibodies144
    16.4.IgG Fc region sialylation and the inhibitory Fc receptor (FcgRIIB)145
    16.5.Cytokine modulation146
    16.6.Neonatal Fc receptor (FcRn)147
    16.7.Dendritic cells (DCs) and immunomodulation148
    16.8.Current and emerging IVIg alternatives and mimetics for use in ITP149
    16.9.Other mechanisms and conclusions151
    17.Immune thrombocytopenia – the patients' perspective158
    17.1.Patients “hate steroids”158
    17.2.Rituximab159
    17.3.Patients try to avoid splenectomy159
    17.4.Patients and Thrombopoetin-Receptor Agonists (TRAs)159
    17.5.Other therapies160
    17.6.The quagmire of modern medicine160
    17.7.What patients really want162
    17.8.Conclusions162
    18.Quality of Life as a treatment endpoint for patients with ITP164
    18.1.Introduction164
    18.2.Assessment of Health Related Quality of Life in ITP164
    18.2.1.HRQoL in Adults164
    18.2.2.HRQoL in Children165
    18.3.ITP symptoms in HRQoL165
    18.3.1.Bleeding and Bruising166
    18.3.2.Fatigue167
    18.4.Impact of treatments on HRQoL167
    18.4.1.First-line agents167
    18.4.2.Second-line therapies168
    18.5.Conclusion169
    19.Fatigue as a treatment end point in ITP172
    19.1.How do we define fatigue172
    19.2.How can we assess fatigue172
    19.3.How much of a problem is fatigue in ITP173
    19.4.Why does fatigue develop in ITP176
    19.5.Cytokines and Immune dysregulation177
    19.6.How do we manage the ITP patient with fatigue179
    19.7.Improving the Standard of Care180
    19.8.Conclusion181
    20.Guidelines for using ITP management guidelines186
    20.1.Guidelines for management of ITP187
    20.2.Pros and cons of guidelines187
    20.3.Evidence based vs. patient centered management188
    21.State of the Art and future Considerations of ITP190
    21.1.Heterogeneity of ITP190
    21.2.Unknown etiology of ITP192
    21.3.Immunomodulation in inflammatory and autoimmune disorders192
    21.4.Platelet stimulation by thrombopoietin receptor agonists (TPO-RA) of megakaryopoiesis193
    21.5.Future progress and the question of adequate management of ITP194
    22.List of abbreviations201
    Index203