{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T01:34:30Z","timestamp":1791941670000,"version":"4.3.4"},"reference-count":35,"publisher":"Japanese Society of Internal Medicine","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Intern. Med."],"published-print":{"date-parts":[[2017]]},"DOI":"10.2169\/internalmedicine.56.7991","type":"journal-article","created":{"date-parts":[[2017,5,14]],"date-time":"2017-05-14T22:11:38Z","timestamp":1494799898000},"page":"1247-1252","source":"Crossref","is-referenced-by-count":21,"title":["Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis\/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging"],"prefix":"10.2169","volume":"56","author":[{"given":"Mizuho","family":"Nara","sequence":"first","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Komatsuda","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumiko","family":"Itoh","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hajime","family":"Kaga","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaya","family":"Saitoh","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaru","family":"Togashi","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiro","family":"Kameoka","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideki","family":"Wakui","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"},{"name":"Department of Life Science, Akita University Graduate School of Engineering Science, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoto","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"962","reference":[{"key":"1","doi-asserted-by":"crossref","unstructured":"1. Soumerai JD, Sohani AR, Abramson JS. Diagnosis and management of Castleman disease. Cancer Control 21: 266-278, 2014.","DOI":"10.1177\/107327481402100403"},{"key":"2","doi-asserted-by":"crossref","unstructured":"2. Casper C, Teltsch DY, Robinson D Jr, et al. Clinical characteristics and healthcare utilization of patients with multicentric Castleman disease. Br J Haematol 168: 82-93, 2015.","DOI":"10.1111\/bjh.13111"},{"key":"3","unstructured":"3. Takai K, Nikkuni K, Shibuya H, Hashidate H. Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly. Rinsyo Ketsueki 51: 320-325, 2010 (in Japanese, Abstract in English)."},{"key":"4","unstructured":"4. Kawabata H, Takai K, Kojima M, et al. Castleman-Kojima disease (TAFRO syndrome): a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly: a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012). J Clin Exp Hematop 53: 57-61, 2013."},{"key":"5","unstructured":"5. Takai K, Nikkuni K, Momoi A, Nagai K, Igarashi N, Saeki S. Thrombocytopenia with reticulin fibrosis accompanied by fever, anasarca and hepatosplenomegaly: a clinical report five cases. J Clin Exp Hematop 53: 63-68, 2013."},{"key":"6","doi-asserted-by":"crossref","unstructured":"6. Masaki Y, Nakajima A, Iwao H, et al. Japanese variant of multicentric Castleman's disease associated with serositis and thrombocytopenia - a report of two cases: Is TAFRO syndrome (Castleman-Kojima disease) a distinct clinicopathological entity? J Clin Exp Hematop 53: 79-85, 2013.","DOI":"10.3960\/jslrt.53.79"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7. Iwaki N, Sato Y, Tanaka K, et al. Atypical hyaline vascular-type Castleman's disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy. J Clin Exp Hematop 53: 87-93, 2013.","DOI":"10.3960\/jslrt.53.87"},{"key":"8","unstructured":"8. Inoue M, Ankou M, Hua J, Iwaki Y, Hagihara M, Ota Y. Complete resolution of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) after immunosuppressive therapies using corticosteroids and cyclosporin A: a case report. J Clin Exp Hematop 53: 95-99, 2013."},{"key":"9","doi-asserted-by":"crossref","unstructured":"9. Awano N, Inomata M, Sonoda Y, et al. A case of multicentric Castleman' disease of mixed-type, which showed constellation of symptoms, i.e., thrombocytopenia, anasarca, anemia, fever, myelofibrosis, and lymphadenopathy. J Clin Exp Hematop 53: 101-105, 2013.","DOI":"10.3960\/jslrt.53.101"},{"key":"10","doi-asserted-by":"crossref","unstructured":"10. Kawabata H, Kotani S, Matsumura Y, et al. Successful treatment of a patient with multicentric Castleman's disease who presented with thrombocytopenia, ascites, renal failure and myelofibrosis using tocilizumab, an anti-interleukin-6 receptor antibody. Intern Med 52: 1503-1507, 2013.","DOI":"10.2169\/internalmedicine.52.9482"},{"key":"11","unstructured":"11. Ozawa T, Kosugi S, Kito M, et al. Efficacy of rituximab for a variant type of multicentric Castleman's disease termed the TAFRO syndrome. Rinsyo Ketsueki 55: 350-355, 2014 (in Japanese, Abstract in English)."},{"key":"12","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1186\/1471-2431-14-139","volume":"14","author":"I Kubokawa","year":"2014","unstructured":"12. Kubokawa I, Yachie A, Hayakawa A, et al. The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman's disease. BMC Pediatrics 14: 139, 2014.","journal-title":"BMC Pediatrics","ISSN":"https:\/\/id.crossref.org\/issn\/1471-2431","issn-type":"print"},{"key":"13","doi-asserted-by":"crossref","unstructured":"13. Koduri PR, Parvez M, Kaza S, Pappu P, Anuradha S. Castleman-Kojima disease in a south Asian adolescent. J Clin Exp Hematop 54: 163-166, 2014.","DOI":"10.3960\/jslrt.54.163"},{"key":"14","doi-asserted-by":"crossref","unstructured":"14. Tatekawa S, Umemura K, Fukuyama R, et al. Thalidomide for tocilizumab-resistant ascites TAFRO syndrome. Clin Case Rep 3: 472-478, 2015.","DOI":"10.1002\/ccr3.284"},{"key":"15","unstructured":"15. Konishi Y, Takahashi S, Nishi K, et al. Successful treatment of TAFRO syndrome, a variant of multicentric Castleman's disease, with cyclosporine A: possible pathogenetic contribution of interleukin-2. Tohoku J Exp Med 236: 289-295, 2015."},{"key":"16","doi-asserted-by":"crossref","unstructured":"16. Jain P, Verstovsek S, Loghavi S, et al. Durable remission with rituximab in a patient with an usual variant of Castleman's disease with myelofibrosis - TAFRO syndrome. Am J Hematol 90: 1091-1092, 2015.","DOI":"10.1002\/ajh.24015"},{"key":"17","doi-asserted-by":"crossref","unstructured":"17. Tedesco S, Postacchini L, Manfredi L, et al. Successful treatment of a Caucasian case of multifocal Castleman's disease with TAFRO syndrome with a pathophysiology targeted therapy - a case report. Exp Hematol Oncol 4: 3, 2015.","DOI":"10.1186\/2162-3619-4-3"},{"key":"18","unstructured":"18. Allegra A, Rotondo F, Russo S, Calabr\u00f2 L, Maisano V. Castleman-Kojima disease (TAFRO syndrome) in a Caucasian patient: a rare case report and review of the literature. Blood Cells Mol Dis 55: 206-207, 2015."},{"key":"19","unstructured":"19. Nagai T, Maemori M, Ando S, et al. Successful treatment with rituximab in tocilizumab-resistant TAFRO syndrome (translation of Japanese title). Rinsyo Ketsueki 56: 62, 2015 (in Japanese, Abstract in English)."},{"key":"20","doi-asserted-by":"crossref","unstructured":"20. Iwaki N, Fajgenbaum DC, Nabel CS, et al. Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease. Am J Hematol 91: 220-226, 2016.","DOI":"10.1002\/ajh.24242"},{"key":"21","doi-asserted-by":"crossref","unstructured":"21. Yamaga Y, Tokuyama K, Kato T, et al. Successful treatment with cyclosporin A in tocilizumab-resistant TAFRO syndrome. Intern Med 55: 185-190, 2016.","DOI":"10.2169\/internalmedicine.55.4710"},{"key":"22","unstructured":"22. Jouvray M, Terriou L, Meignin V, et al. Pseudo-adult Still's disease, anasarca, thrombotic thrombocytopenic purpura and dysautonomia: an atypical presentation of multicentric Castleman's disease. Discussion of TAFRO syndrome. Rev Med Interne 37: 53-57, 2016 (in French, Abctract in English)."},{"key":"23","unstructured":"23. Kawashima M, Usui T, Okada H, et al. TAFRO syndrome: 2 cases and review of the literature. Mod Rheumatol Jul 20: 1-5, 2015 (Epub ahead of print)."},{"key":"24","doi-asserted-by":"crossref","unstructured":"24. Fujiwara S, Mochinaga H, Nakata H, et al. Successful treatment of TAFRO syndrome, a variant type of multicentric Castleman disease with thrombotic microangiopathy, with anti-IL-6 receptor antibody and steroids. Int J Hematol 103: 718-723, 2016.","DOI":"10.1007\/s12185-016-1978-2"},{"key":"25","unstructured":"25. Sakai K, Maeda T, Kuriyama A, Shimada N, Notohara K, Ueda Y. TAFRO syndrome successfully treated with tocilizumab: a case report and systematic review. Mod Rheumatol Feb 17: 1-6, 2016 (Epub ahead of print)."},{"key":"26","doi-asserted-by":"crossref","unstructured":"26. Masaki Y, Kawabata H, Takai K, et al. Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version. Int J Hematol 103: 686-692, 2016.","DOI":"10.1007\/s12185-016-1979-1"},{"key":"27","doi-asserted-by":"crossref","unstructured":"27. Xarli VP, Steele AA, Davis PJ, Buescher ES, Rios CN, Garcia-Bunuel R. Adrenal hemorrhage in the adult. Medicine (Baltimore) 57: 211-221, 1978.","DOI":"10.1097\/00005792-197805000-00002"},{"key":"28","doi-asserted-by":"crossref","unstructured":"28. Fujishima N, Komatsuda A, Ohyagi H, et al. Adrenal insufficiency complicated with antiphospholipid syndrome (APS). Intern Med 45: 963-966, 2006.","DOI":"10.2169\/internalmedicine.45.1603"},{"key":"29","unstructured":"29. Dhawan N, Bodukam VK, Thakur K, Singh A, Jenkins D, Bahl J. Idiopathic bilateral adrenal hemorrhage in a 63-year-old male: a case report and review of the literature. Case Rep Urol 2015: 503638, 2015."},{"key":"30","unstructured":"30. Debatin JF, Spritzer CE, Dunnick NR. Castleman disease of the adrenal gland: MR imaging features. Am J Rent 157: 781-783, 1991."},{"key":"31","doi-asserted-by":"crossref","unstructured":"31. M\u00fcssig K, Gallwitz B, Machicao F, Horger M, H\u00e4ring HU, Kaiserling E. Paraadrenal Castleman disease presenting with adrenal hyperandrogenism. J Endocrinol Invest 29: 172-176, 2006.","DOI":"10.1007\/BF03344093"},{"key":"32","doi-asserted-by":"crossref","unstructured":"32. Otto M, Wieprzowski L, Dzwonkowski J, Ziarkiewicz-Wr\u00f3blewska B. Castleman's disease - an usual indication for laparoscopic adrenalectomy. Wideochir Inne Tech Maloinwazyine 7: 50-54, 2012.","DOI":"10.5114\/wiitm.2011.25620"},{"key":"33","doi-asserted-by":"crossref","unstructured":"33. Limper M, de Kruif MD, Duits AJ, Brandjes DMP, van Gorp ECM. The diagnostic role of procalcitonin and other biomarkers in discriminating infectious from non-infectious fever. J Infect 60: 409-416, 2010.","DOI":"10.1016\/j.jinf.2010.03.016"},{"key":"34","unstructured":"34. Wacker C, Prkno A, Brunkhorst FM, Sclattmann P. Procalcitonin as a diagnostic marker for sepsis: a systemic review and meta-analysis. Lancet Infect Dis 13: 426-435, 2013."},{"key":"35","doi-asserted-by":"crossref","unstructured":"35. Komatsuda A, Omokawa A, Fujiwara T, et al. Serum procalcitonin levels in patients with myeloperoxidase-antineutrophil cytoplasmic antibodies-associated glomerulonephritis. Am J Med Sci 343: 136-140, 2012.","DOI":"10.1097\/MAJ.0b013e31822846db"}],"container-title":["Internal Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/internalmedicine\/56\/10\/56_56.7991\/_pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,9,24]],"date-time":"2019-09-24T16:39:50Z","timestamp":1569343190000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/internalmedicine\/56\/10\/56_56.7991\/_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017]]},"references-count":35,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2017]]}},"URL":"https:\/\/doi.org\/10.2169\/internalmedicine.56.7991","relation":{},"ISSN":["0918-2918","1349-7235"],"issn-type":[{"value":"0918-2918","type":"print"},{"value":"1349-7235","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017]]}}}