Unicentric castleman disease: Updates and novel insights into spindle cell proliferations and aggressive forms of a localized disease Review


Authors: Alnoor, F. N. U.; Rangel, A.; Luo, M.; Silva, O.; Chisholm, K. M.; O'Malley, D.; Warnke, R.; Kumar, J.; Ohgami, R. S.
Review Title: Unicentric castleman disease: Updates and novel insights into spindle cell proliferations and aggressive forms of a localized disease
Abstract: Castleman Disease (CD) is a rare lymphoproliferative disorder that can be separated into two primary forms: Unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD is localized, while MCD is systemic. Though UCD generally has a favorable prognosis following surgical resection, more aggressive forms of this disease have been identified, including cases associated with dendritic and spindle cell proliferation. Genetic analysis has deepened our understanding of UCD. Despite advancements in better understanding the pathophysiology of UCD, challenges persist in the diagnosis, management, and treatment due to its rarity and heterogeneity. Here, we review current knowledge on UCD, highlighting the epidemiology, clinical presentation, diagnostic criteria, and treatment options while emphasizing the need for further research and innovation in therapeutic strategies. © 2024 John Wiley & Sons Ltd.
Keywords: review; pathophysiology; genetic analysis; cell proliferation; pathology; retrospective study; diagnosis; interleukin 6; interleukin-6; surgery; spindle cell; lymphoproliferative disease; epidemiology; therapy; etiology; angiofollicular lymph node hyperplasia; pdgfrb; follicular dendritic cells; follicular dendritic cell; humans; prognosis; human; castleman disease; unicentric castleman disease; multicentric castleman disease; immunoglobulin g4 related disease; idiopathic multicentric castleman disease; igg4 related disease
Journal Title: International Journal of Laboratory Hematology
Volume: 47
Issue: 1
ISSN: 1751-5521
Publisher: Wiley Blackwell  
Date Published: 2025-02-01
Start Page: 26
End Page: 35
Language: English
DOI: 10.1111/ijlh.14395
PUBMED: 39501556
PROVIDER: scopus
DOI/URL:
Notes: Review -- Source: Scopus
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  1. Jyoti Kumar
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