Transcriptome and unique cytokine microenvironment of Castleman disease.


Journal

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
ISSN: 1530-0285
Titre abrégé: Mod Pathol
Pays: United States
ID NLM: 8806605

Informations de publication

Date de publication:
04 2022
Historique:
received: 31 07 2021
accepted: 05 10 2021
revised: 05 10 2021
pubmed: 24 10 2021
medline: 29 4 2022
entrez: 23 10 2021
Statut: ppublish

Résumé

Castleman disease (CD) represents a group of rare, heterogeneous and poorly understood disorders that share characteristic histopathological features. Unicentric CD (UCD) typically involves a single enlarged lymph node whereas multicentric CD (MCD) involves multiple lymph node stations. To understand the cellular basis of CD, we undertook a multi-platform analysis using targeted RNA sequencing, RNA in-situ hybridization (ISH), and adaptive immune receptor rearrangements (AIRR) profiling of archived tissue from 26 UCD, 14 MCD, and 31 non-CD reactive controls. UCD showed differential expression and upregulation of follicular dendritic cell markers (CXCL13, clusterin), angiogenesis factors (LPL, DLL4), extracellular matrix remodeling factors (TGFβ, SKIL, LOXL1, IL-1β, ADAM33, CLEC4A), complement components (C3, CR2) and germinal center activation markers (ZDHHC2 and BLK) compared to controls. MCD showed upregulation of IL-6 (IL-6ST, OSMR and LIFR), IL-2, plasma cell differentiation (XBP1), FDC marker (CXCL13, clusterin), fibroblastic reticular cell cytokine (CCL21), angiogenesis factor (VEGF), and mTORC1 pathway genes compared to UCD and controls. ISH studies demonstrated that VEGF was increased in the follicular dendritic cell-predominant atretic follicles and the interfollicular macrophages of MCD compared to UCD and controls. IL-6 expression was higher along interfollicular vasculature-associated cells of MCD. Immune repertoire analysis revealed oligoclonal expansions of T-cell populations in MCD cases (2/6) and UCD cases (1/9) that are consistent with antigen-driven T cell activation. The findings highlight the unique genes, pathways and cell types involved in UCD and MCD. We identify potential novel targets in CD that may be harnessed for therapeutics.

Identifiants

pubmed: 34686774
doi: 10.1038/s41379-021-00950-3
pii: S0893-3952(22)00289-7
pmc: PMC9272352
mid: NIHMS1815112
doi:

Substances chimiques

Clusterin 0
Cytokines 0
Interleukin-6 0
Vascular Endothelial Growth Factor A 0
ADAM Proteins EC 3.4.24.-
ADAM33 protein, human EC 3.4.24.-

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

451-461

Subventions

Organisme : NCI NIH HHS
ID : P30 CA016520
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL141408
Pays : United States

Informations de copyright

© 2021. The Author(s), under exclusive licence to United States & Canadian Academy of Pathology.

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Auteurs

Anna Wing (A)

Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Jason Xu (J)

Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Wenzhao Meng (W)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.

Aaron M Rosenfeld (AM)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.

Elizabeth Y Li (EY)

Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Gerald Wertheim (G)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.
Division of Hematopathology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Michele Paessler (M)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.
Division of Hematopathology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Adam Bagg (A)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.

Dale Frank (D)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.

Kai Tan (K)

Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

David T Teachey (DT)

Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Megan S Lim (MS)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.

Eline Luning Prak (EL)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.

David C Fajgenbaum (DC)

Center for Cytokine Storm Treatment and Laboratory, Department of Medicine, University of Pennsylvania, Philadelphia, USA.

Vinodh Pillai (V)

Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA. pillaiv1@chop.edu.
Division of Hematopathology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. pillaiv1@chop.edu.

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