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Loss of interleukin-21 leads to atrophic germinal centers in multicentric Castleman’s disease

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Abstract

Both multicentric Castleman’s disease (MCD) and immunoglobulin (Ig)G4-related disease (IgG4-RD) are systemic diseases, presenting with hypergammaglobulinemia and elevated serum levels of IgG4. However, with regard to histopathological findings, MCD shows atrophic germinal centers. On the other hand, expanded germinal centers are detected in IgG4-RD. We extracted germinal centers from specimens of each disorder by microdissection and analyzed the expression of mRNAs by real-time polymerase chain reaction to clarify the mechanisms underlying atrophied germinal centers in MCD. This analysis disclosed loss of interleukin (IL)-21 and B cell lymphoma (Bcl)-6 in the germinal centers of MCD. Loss of IL-21 is considered to be involved in the disappearance of Bcl-6 and leads to atrophied germinal centers in MCD.

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Acknowledgments

This work was supported by the Research on Measures for Intractable Diseases Project matching fund subsidy from the Ministry of Health Labour and Welfare, Japan, and the Minako Shiokawa Young Investor’s Award for Collagen Disease Research, from the Japan Rheumatism Foundation.

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Division of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, South 1 West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan

    Hidetaka Yajima, Motohisa Yamamoto, Yui Shimizu, Nodoka Sakurai, Chisako Suzuki, Yasuyoshi Naishiro, Yasuhisa Shinomura & Hiroki Takahashi

  2. Department of Internal Medicine and Gastroenterlogy, Sapporo Dohkohkai Hospital, Sapporo, Japan

    Hidetaka Yajima

  3. Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Japan

    Nodoka Sakurai

  4. The Institute of Medical Science, The University of Tokyo, Tokyo, Japan

    Kohzoh Imai

  5. Ikeda Municipal Hospital, Osaka, Japan

    Yasuhisa Shinomura

Authors
  1. Hidetaka Yajima
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  2. Motohisa Yamamoto
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  3. Yui Shimizu
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  4. Nodoka Sakurai
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  5. Chisako Suzuki
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  6. Yasuyoshi Naishiro
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  7. Kohzoh Imai
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  8. Yasuhisa Shinomura
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  9. Hiroki Takahashi
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Corresponding author

Correspondence to Motohisa Yamamoto.

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Yajima, H., Yamamoto, M., Shimizu, Y. et al. Loss of interleukin-21 leads to atrophic germinal centers in multicentric Castleman’s disease. Ann Hematol 95, 35–40 (2016). https://doi.org/10.1007/s00277-015-2500-2

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  • DOI: https://doi.org/10.1007/s00277-015-2500-2

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