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Castleman Disease, An Issue of Hematology/Oncology Clinics, E-Book

Castleman Disease, An Issue of Hematology/Oncology Clinics, E-Book

Frits van Rhee | Nikhil C. Munshi

(2017)

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Book Details

Abstract

This issue of Hematology/Oncology Clinics, edited by Dr. Frits van Rhee and Dr. Nikhil C. Munshi, focuses on Castleman Disease. Topics include, but are not limited to, Epidemiology; Pathogenesis; Role of Interleukin 6 in Castleman Disease; Pathology, Diagnosis; Unicentric Castleman Disease; Treatment of HHV8 related Multicentric Castleman Disease; Treatment of HHV8 negative Castleman Disease; TAFRO Syndrome; POEMS; and more.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Castleman Disease\r i
Copyright\r ii
Contributors iii
CONSULTING EDITORS iii
EDITORS iii
AUTHORS iii
Contents vii
Preface: Castleman Disease vii
Epidemiology of Castleman Disease vii
Castleman Disease Pathogenesis vii
The Role of Interleukin-6 in Castleman Disease vii
Pathology of Castleman Disease viii
Diagnosis of Castleman Disease viii
Unicentric Castleman Disease viii
Treatment of Kaposi Sarcoma Herpesvirus–Associated Multicentric Castleman Disease viii
Treatment of Idiopathic Castleman Disease ix
TAFRO Syndrome ix
POEMS Syndrome: Diagnosis and Investigative Work-up ix
POEMS Syndrome: Therapeutic Options ix
The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease x
HEMATOLOGY/ONCOLOGY\rCLINICS OF NORTH AMERICA\r xi
FORTHCOMING ISSUES xi
April 2018 xi
June 2018 xi
August 2018 xi
RECENT ISSUES xi
December 2017 xi
October 2017 xi
August 2017 xi
Preface:\rCastleman Disease xiii
REFERENCE xiv
Epidemiology of Castleman Disease 1
Key points 1
UNICENTRIC CASTLEMAN DISEASE 2
HUMAN HERPESVIRUS-8 PLUS MULTICENTRIC CASTLEMAN DISEASE 4
IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE 5
POLYNESIAN IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE 5
POLYRADICULONEUROPATHY, ORGANOMEGALY, ENDOCRINOPATHY, MONOCLONAL PLASMA CELL NEOPLASM, AND SKIN CHANGES RELATED TO IDIOPATH ... 6
THROMBOCYTOPENIA, ANASARCA, FEVER, RENAL INSUFFICIENCY, AND ORGANOMEGALY 7
SUMMARY 7
REFERENCES 8
Castleman Disease Pathogenesis 11
Key points 11
INTRODUCTION 11
UNICENTRIC CASTLEMAN DISEASE 13
Cause 13
Cell Type 13
Signaling Pathways 13
Effector Cytokines 14
HUMAN HERPESVIRUS-8–ASSOCIATED MULTICENTRIC CASTLEMAN DISEASE 14
Cause 14
Cell Type 14
Signaling Pathways 14
Effector Cytokines 15
HUMAN HERPESVIRUS-8–NEGATIVE MULTICENTRIC CASTLEMAN DISEASE 15
POEMS–Associated Multicentric Castleman Disease 15
Cause/cell type 15
Signaling pathways/effector cytokines 15
Idiopathic Multicentric Castleman Disease 15
Cause 15
Autoimmune 15
Autoinflammatory 16
Neoplastic 16
Pathogen 16
Cell type 17
Signaling pathways/effector cytokines 17
FUTURE DIRECTIONS 18
REFERENCES 18
The Role of Interleukin-6 in Castleman Disease 23
Key points 23
INTRODUCTION 23
DISCOVERY, PRODUCTION, AND FUNCTION OF INTERLEUKIN-6 24
INTERLEUKIN-6 RECEPTOR SYSTEM AND SIGNAL TRANSDUCTION PATHWAY 25
REVELATION OF THE ROLE OF INTERLEUKIN-6 IN THE PATHOGENESIS OF CASTLEMAN DISEASE 25
BLOCKING INTERLEUKIN-6 FUNCTION WITH A HUMANIZED ANTI-INTERLEUKIN-6 RECEPTOR ANTIBODY 28
CLINICAL USE OF A HUMANIZED ANTI-INTERLEUKIN-6R ANTIBODY 29
CLINICAL STUDY OF TOCILIZUMAB OR SILTUXIMAB FOR MULTICENTRIC CASTLEMAN DISEASE THERAPY 30
SUMMARY 33
ACKNOWLEDGMENTS 35
REFERENCES 35
Pathology of Castleman Disease 37
Key points 37
INTRODUCTION 37
HISTOPATHOLOGIC FEATURES 41
Unicentric Castleman Disease 41
Multicentric Castleman Disease 43
Hypervascular variant of multicentric disease 43
Plasmacytic variant of multicentric disease 44
Thrombocytopenia, ascites/anasarca, myelofibrosis/fever, renal dysfunction/reticulin fibrosis, and organomegaly syndrome 45
Human Herpes Virus 8-Positive, Castleman Disease 45
HISTOLOGIC DIFFERENTIAL DIAGNOSES 46
CONSENSUS DIAGNOSTIC CRITERIA FOR HUMAN HERPES VIRUS 8-NEGATIVE, IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE 47
SUMMARY AND DISCUSSION 48
REFERENCES 49
Diagnosis of Castleman Disease 53
Key points 53
PRESENTATION 54
HISTOPATHOLOGICAL EXAMINATION OF CASTLEMAN DISEASE 54
LABORATORY AND RADIOLOGICAL INVESTIGATIONS 55
UNICENTRIC CASTLEMAN DISEASE 55
HUMAN HERPESVIRUS 8 AND HUMAN IMMUNODEFICIENCY VIRUS-POSITIVE MULTICENTRIC CASTLEMAN DISEASE 57
MULTICENTRIC CASTLEMAN DISEASE HUMAN HERPESVIRUS 8-POSITIVE HUMAN IMMUNODEFICIENCY VIRUS-NEGATIVE 57
IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE 57
SPECIFIC PRESENTATIONS OF CASTLEMAN DISEASE 59
Paraneoplastic Pemphigus 59
POEMS 59
TAFRO 61
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS 61
AUTOIMMUNE CYTOPENIA 61
PERIPHERAL NEUROPATHY 61
RENAL INVOLVEMENT 62
DIFFERENTIAL DIAGNOSIS 62
REFERENCES 62
Unicentric Castleman Disease 65
Key points 65
INTRODUCTION 65
CLINICAL MANIFESTATIONS 66
INVESTIGATIONS AND DIAGNOSIS 66
Plain Radiographs 66
Computed Tomography 66
MRI 67
Ultrasonography 67
PET 67
Laboratory Studies 68
TREATMENT 68
Surgery 68
Radiotherapy 68
Other Therapeutic Options 68
PROGNOSIS 69
DISEASES ASSOCIATED WITH UNICENTRIC CASTLEMAN DISEASE 69
Paraneoplastic Pemphigus 69
Lymphoma 69
Follicular Dendritic Cell Sarcoma 69
SUMMARY 69
REFERENCES 70
Treatment of Kaposi Sarcoma Herpesvirus–Associated Multicentric Castleman Disease 75
Key points 75
INTRODUCTION 75
EPIDEMIOLOGY 76
PATHOGENESIS 76
DIAGNOSIS 77
TREATMENT 79
SUMMARY 84
DISCLOSURES 84
ACKNOWLEDGMENTS 85
REFERENCES 85
Treatment of Idiopathic Castleman Disease 89
Key points 89
INTRODUCTION 89
CLINICAL SYMPTOMATOLOGY OF MULTICENTRIC CASTLEMAN DISEASE AND ROLE OF INTERLEUKIN-6 90
Multicentric Castleman Disease Classification and Therapy 91
Therapeutic Options for Idiopathic Multicentric Castleman Disease 92
Overview 92
Overall outcome of idiopathic multicentric Castleman disease 92
Surgery and irradiation 93
Corticosteroids 93
Chemotherapy 93
Rituximab 94
Anti-IL6 agents 94
Immunomodulatory agents 97
Treatment Guidelines and Algorithm 98
SUMMARY 100
REFERENCES 100
TAFRO Syndrome 107
Key points 107
INTRODUCTION 107
CLINICAL CHARACTERISTICS 109
Age at Onset 109
Symptoms 109
Laboratory Findings 109
HISTOPATHOLOGICAL CHARACTERISTICS 110
Lymph Nodes 110
Bone Marrow 110
DIAGNOSIS 110
The Need for Differential Diagnosis 110
Proposed Diagnostic Criteria 111
Importance of Lymph Node Biopsy 113
PROGNOSIS AND CLASSIFICATION OF SEVERITY 113
DISEASE MECHANISMS 114
The Role of Interleukin-6 114
Platelet Count Depletion 114
Is Bacterial Infection a Cause of TAFRO Syndrome? 114
CLINICAL MANAGEMENT 115
SUMMARY 115
REFERENCES 116
POEMS Syndrome 119
Key points 119
INTRODUCTION 119
HISTORY 120
PATHOPHYSIOLOGY 121
PERIPHERAL NEUROPATHY 121
MONOCLONAL PLASMA CELL DISORDER AND HEMATOLOGIC FINDINGS 123
BONE LESIONS 124
EXTRAVASCULAR VOLUME OVERLOAD 124
VASCULAR ENDOTHELIAL GROWTH FACTOR AND OTHER CYTOKINES 125
ORGANOMEGALY 125
ENDOCRINOPATHY 125
SKIN FINDINGS 125
PAPILLEDEMA 126
RESPIRATORY FINDINGS 126
RENAL FINDINGS 126
THROMBOSIS 127
OTHER LABORATORY FINDINGS 128
DIFFERENTIAL DIAGNOSIS 128
TESTING GUIDELINES 130
SUMMARY POINTS 130
REFERENCES 130
POEMS Syndrome 141
Key points 141
INTRODUCTION 141
UNDERLYING HEMOPATHY 142
SPECIFIC TREATMENTS 142
TREATMENT WHEN THERE ARE 1 TO 3 BONE LESIONS AND NO DISSEMINATED BONE MARROW INVOLVEMENT 143
TREATMENT WHEN THERE IS DISSEMINATED BONE MARROW INVOLVEMENT OR NO BONE LESION OR MANY BONE LESIONS 144
SECOND-LINE TREATMENT 147
MONITORING RESPONSE 148
PROGNOSIS 148
SUPPORTING TREATMENT 149
CONCLUDING REMARKS 149
REFERENCES 149
The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease 153
Key points 153
INTRODUCTION 153
POLYNEUROPATHY, ORGANOMEGALY, ENDOCRINOPATHY, MONOCLONAL PLASMA CELL-PROLIFERATIVE DISORDER, SKIN CHANGES (POEMS) SYNDROME 154
General 154
Neuropathy Characteristics 155
Evaluation 155
Electrodiagnostic Characteristics 156
Radiologic Features 156
Pathologic Features 157
Treatment 157
CASTLEMAN DISEASE 159
General 159
Neuropathy Characteristics 160
Evaluation 160
Electrophysiologic Features 160
Pathologic Features 161
Treatment 161
SUMMARY 161
REFERENCES 161