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International Journal of Hematology

, Volume 103, Issue 6, pp 686–692 | Cite as

Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version

  • Yasufumi MasakiEmail author
  • Hiroshi Kawabata
  • Kazue Takai
  • Masaru Kojima
  • Norifumi Tsukamoto
  • Yasuhito Ishigaki
  • Nozomu Kurose
  • Makoto Ide
  • Jun Murakami
  • Kenji Nara
  • Hiroshi Yamamoto
  • Yoko Ozawa
  • Hidekazu Takahashi
  • Katsuhiro Miura
  • Tsutomu Miyauchi
  • Shinichirou Yoshida
  • Akihito Momoi
  • Nobuyasu Awano
  • Soichiro Ikushima
  • Yasunori Ohta
  • Natsue Furuta
  • Shino Fujimoto
  • Haruka Kawanami
  • Tomoyuki Sakai
  • Takafumi Kawanami
  • Yoshimasa Fujita
  • Toshihiro Fukushima
  • Shigeo Nakamura
  • Tomohiro Kinoshita
  • Sadao Aoki
Original Article

Abstract

TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca including pleural effusion and ascites, fever, renal insufficiency, and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology is undetermined. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of multicentric Castleman disease (MCD), other specific features can differentiate between them. Some TAFRO syndrome patients have been successfully treated with glucocorticoids and/or immunosuppressants, including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, and eventually succumb to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The present article reports the 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams. These criteria and classification have been applied and retrospectively validated on clinicopathologic data of 28 patients with this and similar conditions (e.g. MCD with serositis and thrombocytopenia).

Keywords

Thrombocytopenia Anasarca Glucocorticoid Cyclosporin A Tocilizumab 

Notes

Acknowledgments

We thank all participants in the All Japan, Ministry of Health, Labor, and Welfare (MHLW) TAFRO Syndrome Team and Castleman Disease Team including for their critical discussion.

Compliance with ethical standards

Supportive foundations

Sources of support in the form of grants: 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-Nanchi, etc.(Nan)-General-008), and by the Japanese 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).

Conflict of interest

The authors declare there is no conflict of interest.

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Copyright information

© The Japanese Society of Hematology 2016

Authors and Affiliations

  • Yasufumi Masaki
    • 1
    Email author
  • Hiroshi Kawabata
    • 2
  • Kazue Takai
    • 3
  • Masaru Kojima
    • 4
  • Norifumi Tsukamoto
    • 5
  • Yasuhito Ishigaki
    • 6
  • Nozomu Kurose
    • 7
  • Makoto Ide
    • 8
  • Jun Murakami
    • 9
  • Kenji Nara
    • 10
  • Hiroshi Yamamoto
    • 11
  • Yoko Ozawa
    • 11
  • Hidekazu Takahashi
    • 11
  • Katsuhiro Miura
    • 12
  • Tsutomu Miyauchi
    • 13
  • Shinichirou Yoshida
    • 14
  • Akihito Momoi
    • 15
  • Nobuyasu Awano
    • 16
  • Soichiro Ikushima
    • 16
  • Yasunori Ohta
    • 17
  • Natsue Furuta
    • 18
  • Shino Fujimoto
    • 1
  • Haruka Kawanami
    • 1
  • Tomoyuki Sakai
    • 1
  • Takafumi Kawanami
    • 1
  • Yoshimasa Fujita
    • 1
  • Toshihiro Fukushima
    • 1
  • Shigeo Nakamura
    • 19
  • Tomohiro Kinoshita
    • 20
  • Sadao Aoki
    • 21
  1. 1.Division of Hematology and Immunology, MedicineKanazawa Medical UniversityKahokuJapan
  2. 2.Department of Hematology and Oncology, Graduate School of MedicineKyoto UniversityKyotoJapan
  3. 3.Division of HematologyNiigata City General HospitalNiigataJapan
  4. 4.Department of Diagnostic PathologyDokkyo University School of MedicineTochigiJapan
  5. 5.Department of Medicine and Clinical ScienceGunma University Graduate School of MedicineMaebashiJapan
  6. 6.Medical Research InstituteKanazawa Medical UniversityUchinadaJapan
  7. 7.Department of Pathology and Laboratory MedicineKanazawa Medical UniversityUchinadaJapan
  8. 8.Department of HematologyTakamatsu Red Cross HospitalTakamatsuJapan
  9. 9.Department of Gastroenterology and HematologyUniversity of ToyamaToyamaJapan
  10. 10.Department of HematologySeirei Mikatahara General HospitalHamamatsuJapan
  11. 11.1st Department of Internal MedicineShinshu University School of MedicineNaganoJapan
  12. 12.Department of Hematology and RheumatologyNihon University School of MedicineTokyoJapan
  13. 13.Toyama PET Imaging CenterToyamaJapan
  14. 14.Department of HematologyNational Hospital Organization Nagasaki Medical CenterNagasakiJapan
  15. 15.Department of HematologyNiigata Prefectural Central HospitalNiigataJapan
  16. 16.Department of Respiratory MedicineJapanese Red Cross Medical CenterTokyoJapan
  17. 17.Department of PathologyTokyo University HospitalTokyoJapan
  18. 18.Department of Internal MedicineNiigata Cancer Center HospitalNiigataJapan
  19. 19.Department of Pathology and Biological ResponseNagoya University Graduate School of MedicineNagoyaJapan
  20. 20.Department of Hematology and Cell TherapyAichi Cancer Center HospitalNagoyaJapan
  21. 21.Department of Pathophysiology, Faculty of Pharmaceutical SciencesNiigata University of Pharmacy and Applied Life SciencesNiigataJapan

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