The potential role of follicular helper T cells in idiopathic multicentric Castleman disease with and without TAFRO syndrome.

Abstract

Idiopathic multicentric Castleman disease (iMCD) is a systemic inflammatory disease of unknown etiology caused by hypercytokinemia. Recently, TAFRO (thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly) syndrome has been reported, which shows similar histopathological findings to iMCD and factors associated with a poor prognosis. iMCD shows no plasma cell infiltration in the germinal center (GC), but CD38-positive (CD38+)-plasma cells are observed in the interfollicular area. Our previous report revealed that atrophic change of GC, glomeruloid vascular proliferation, and abnormal proliferation of follicular dendritic cells are more prominent in iMCD with TAFRO (TAFRO+) in comparison to iMCD without TAFRO (TAFRO-). In addition, the numbers of CD38+ and immunoglobulin G4-positive (IgG4+) plasma cells were decreased in the interfollicular area. The roles of T follicular helper cells (Tfh) are well-known to assist B-cell proliferation, maturation, and differentiation．It maintains the formation of GC and is also related in the class switching of IgG isotypes, including IgG4. Thus, we immunohistochemically examined the number of Tfh in GCs in both TAFRO- and TAFRO+ iMCD. The number of Tfh was significantly decreased in TAFRO- iMCD (n = 9) and was further decreased in TAFRO+ iMCD (n = 18) in comparison to non-specific lymphadenopathy (n = 6) and IgG4-related disease (n = 4). These results suggest that decreased Tfh may be one etiology of iMCD.

• Authors: Nozomu Kurose, Xin Guo, Akihiro Shioya, Ken-Ichi Mizutani, Motona Kumagai, Shino Fujimoto, Hiroshi Kawabata, Yasufumi Masaki, Kazue Takai, Sadao Aoki, Shigeo Nakamura, Sohsuke Yamada
• Journal: Pathology, research and practice (Pathol Res Pract) Vol. 215 Issue 10 Pg. 152563 (Oct 2019) ISSN: 1618-0631 [Electronic] Germany
• PMID: 31358479 (Publication Type: Journal Article, Multicenter Study)
• Copyright: Copyright © 2019 Elsevier GmbH. All rights reserved.

Chemical References

• Immunoglobulin G

Topics

• Castleman Disease (metabolism, pathology)
• Humans
• Immunoglobulin G (blood)
• Plasma Cells (metabolism, pathology)
• Retrospective Studies
• T-Lymphocytes, Helper-Inducer (metabolism, pathology)