Recent Advances in Castleman Disease
© 2022 S. Karger AG, Basel..
BACKGROUND: Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric CD (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric CD (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpesvirus-8 (HHV-8 + MCD).
SUMMARY: During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD.
KEY MESSAGES: We herein summarize recent advances in diagnosis, treatment, and novel insights into the pathogenesis of this disease.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:45 |
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| Enthalten in: |
Oncology research and treatment - 45(2022), 11 vom: 08., Seite 693-704 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Hoffmann, Christian [VerfasserIn] |
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| Links: |
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| Themen: |
Castleman disease |
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| Anmerkungen: |
Date Completed 02.11.2022 Date Revised 02.11.2022 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1159/000526640 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM347351638 |
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| 520 | |a © 2022 S. Karger AG, Basel. | ||
| 520 | |a BACKGROUND: Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric CD (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric CD (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpesvirus-8 (HHV-8 + MCD) | ||
| 520 | |a SUMMARY: During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD | ||
| 520 | |a KEY MESSAGES: We herein summarize recent advances in diagnosis, treatment, and novel insights into the pathogenesis of this disease | ||
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| 700 | 1 | |a Weber, Florian |e verfasserin |4 aut | |
| 700 | 1 | |a Willenbacher, Wolfgang |e verfasserin |4 aut | |
| 700 | 1 | |a Hübel, Kai |e verfasserin |4 aut | |
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