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Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan

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Abstract

TAFRO syndrome is a systemic inflammatory disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. Mortality in patients with this syndrome is high; however, an optimal treatment strategy has not been established. To explore the strategy, we retrospectively analyzed 81 patients with TAFRO syndrome registered in the Multicenter Collaborative Retrospective Study for Establishing the Concept of TAFRO Syndrome in Japan by December 2019. Sixty-eight patients received corticosteroid therapy as the first-line treatment, and as the second-line treatment, 21 received tocilizumab (Toc), 14 received cyclosporine A (CsA), and 8 received rituximab (Rit) in addition to corticosteroids. We compared these second-line treatment groups by setting the primary endpoint as time to next treatment or death (TTNT). Kaplan–Meier analysis showed that the median TTNT in the Toc, CsA, and Rit groups were 2.8 months, 9.2 months, and not reached, respectively. The TTNT of the Rit group was significantly longer than that of the Toc group. In contrast, there were no significant differences in overall survival between groups, indicating that subsequent salvage therapies rescued a large proportion of patients who failed the second-line treatments. Further studies are warranted to establish the optimal treatment strategies for this syndrome.

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Acknowledgements

The authors thank the patients, their families, all the investigators and nurses in the collaborating centers of this study listed in the Supplementary Table S1. We would like to thank Editage (www.editage.com) for English language editing.

This work was partially supported by the Research Program of Intractable Disease provided by the Ministry of Health, Labor, and Welfare (MHLW) of Japan (H27-28 Nanchi, etc. (Nan)-General-002; H27-Nanchi, etc. (Nan)-General-008; H29-Nanchi, etc. (Nan)-General-008; H29-Nanchi, etc. (Nan)-General-019; H29-Nanchi, etc. (Nan)-General-058), and by the Ministry of Education, Culture, Sports, Science and Technology (Grant no.17591060 and 15K09510), the Kanazawa Medical University Research Foundation (Grant nos. S2004-16 and S2007-5), Grant for Assist KAKEN from Kanazawa Medical University (Grant no.K2011-7), Grant for Project Research from High-Tech Research Center of Kanazawa Medical University (Grant no.H2011-11) and Grant for Alumni Research (A) from Kanazawa Medical University (AR2012-06).

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Authors and Affiliations

  1. Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Ishikawa-ken, 920-0293, Japan

    Shino Fujimoto, Hiroshi Kawabata, Tomoyuki Sakai, Hiroto Yanagisawa & Yasufumi Masaki

  2. Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Kyoto, Japan

    Hiroshi Kawabata & Momoko Nishikori

  3. Department of Hematology, Seirei Mikatahara General Hospital, Hamamatsu, Japan

    Kenji Nara

  4. Department of Hematology, Eiju General Hospital, Tokyo, Japan

    Shin Ohara

  5. Oncology Centre, Gunma University Hospital, Maebashi, Japan

    Norifumi Tsukamoto

  6. Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Uchinada, Japan

    Nozomu Kurose & Sohsuke Yamada

  7. Department of Hematology, Niigata City General Hospital, Niigata, Japan

    Kazue Takai

  8. Department of Pathophysiology, Faculty of Pharmaceutical Sciences, Niigata University of Pharmacy and Applied Life Sciences, Niigata, Japan

    Sadao Aoki

Authors
  1. Shino Fujimoto
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  2. Hiroshi Kawabata
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  3. Tomoyuki Sakai
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  4. Hiroto Yanagisawa
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  6. Kenji Nara
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  7. Shin Ohara
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  8. Norifumi Tsukamoto
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  9. Nozomu Kurose
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  10. Sohsuke Yamada
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  11. Kazue Takai
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  12. Sadao Aoki
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  13. Yasufumi Masaki
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Corresponding author

Correspondence to Hiroshi Kawabata.

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Conflict of interest

Dr. Kawabata reports grants from Ministry of Health, Labor, and Welfare (MHLW) of Japan, during the conduct of the study. Dr. Nishikori reports personal fees from Eisai Co., Ltd., grants from Eisai Co., Ltd., grants from Sumitomo Dainippon Pharma Co., Ltd., outside the submitted work. Dr. Tsukamoto reports scholarship donation from Kyowa Hakko Kirin Co., Ltd., outside the submitted work. Dr. Aoki reports personal fees from AbbVie GK., personal fees from Janssen Pharmaceutical K.K, outside the submitted work. Dr. Masaki reports grants from Ministry of Health, Labor, and Welfare (MHLW) of Japan, grants from Ministry of Education, Culture, Sports, Science and Technology, grants from Kanazawa Medical University, grants from Eisai Co., Ltd., grants from Kyowa Hakko Kirin Co., Ltd., grants from Astellas Pharma Inc., grants from Ono Pharmaceutical Co. Ltd., grants from Pfizer Seiyaku K. K., during the conduct of the study.

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Fujimoto, S., Kawabata, H., Sakai, T. et al. Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan. Int J Hematol 113, 73–80 (2021). https://doi.org/10.1007/s12185-020-03008-3

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  • DOI: https://doi.org/10.1007/s12185-020-03008-3

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