{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T06:17:33Z","timestamp":1788675453985,"version":"build-2803163510"},"reference-count":30,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T00:00:00Z","timestamp":1768780800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T00:00:00Z","timestamp":1768780800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Int J Hematol"],"published-print":{"date-parts":[[2026,4]]},"DOI":"10.1007\/s12185-025-04147-1","type":"journal-article","created":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T03:38:44Z","timestamp":1768793924000},"page":"617-626","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Ruxolitinib in relapsed\/refractory TAFRO syndrome: a report of two cases, including one positive for Epstein\u2013Barr virus, and a literature review"],"prefix":"10.1007","volume":"123","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5989-7786","authenticated-orcid":false,"given":"Akihisa","family":"Kawajiri","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenta","family":"Takenaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiro","family":"Narumi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makiko","family":"Tsukita","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rie","family":"Shibuya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Hashimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joji","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,1,19]]},"reference":[{"key":"4147_CR1","doi-asserted-by":"publisher","first-page":"57","DOI":"10.3960\/jslrt.53.57","volume":"53","author":"H Kawabata","year":"2013","unstructured":"Kawabata H, Takai K, Kojima M, Nakamura N, Aoki S, Nakamura S, et al. Castleman-Kojima disease (TAFRO syndrome)\u202f: a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly\u202f: a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012). J Clin Exp Hematopathol. 2013;53:57\u201361.","journal-title":"J Clin Exp Hematopathol"},{"key":"4147_CR2","first-page":"320","volume":"51","author":"K Takai","year":"2010","unstructured":"Takai K, Nikkuni K, Shibuya H, Hashidate H. [Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly]. Rinsho Ketsueki. 2010;51:320\u20135.","journal-title":"Rinsho Ketsueki"},{"key":"4147_CR3","first-page":"102","volume":"2","author":"L Abdo","year":"2014","unstructured":"Abdo L. First European case of TAFRO syndrome associated with Sjogren disease. Am J Intern Med. 2014;2:102\u20135.","journal-title":"Am J Intern Med"},{"key":"4147_CR4","doi-asserted-by":"publisher","DOI":"10.1186\/2162-3619-4-3","volume":"4","author":"S Tedesco","year":"2015","unstructured":"Tedesco S, Postacchini L, Manfredi L, Goteri G, Luchetti MM, Festa A, et al. Successful treatment of a Caucasian case of multifocal Castleman\u2019s disease with TAFRO syndrome with a pathophysiology targeted therapy - a case report. Exp Hematol Oncol. 2015;4:3.","journal-title":"Exp Hematol Oncol"},{"key":"4147_CR5","doi-asserted-by":"publisher","first-page":"220","DOI":"10.1002\/ajh.24242","volume":"91","author":"N Iwaki","year":"2016","unstructured":"Iwaki N, Fajgenbaum DC, Nabel CS, Gion Y, Kondo E, Kawano M, et al. Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease. Am J Hematol. 2016;91:220\u20136.","journal-title":"Am J Hematol"},{"key":"4147_CR6","doi-asserted-by":"publisher","first-page":"27","DOI":"10.2169\/internalmedicine.9622-22","volume":"62","author":"Y Masaki","year":"2023","unstructured":"Masaki Y, Ueda Y, Yanagisawa H, Arita K, Sakai T, Yamada K, et al. Tafro syndrome: a disease requiring immediate medical attention. Intern Med. 2023;62:27\u201332.","journal-title":"Intern Med"},{"key":"4147_CR7","doi-asserted-by":"publisher","first-page":"1241","DOI":"10.1002\/ajh.26292","volume":"96","author":"Y Nishimura","year":"2021","unstructured":"Nishimura Y, Fajgenbaum DC, Pierson SK, Iwaki N, Nishikori A, Kawano M, et al. Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease. Am J Hematol. 2021;96:1241\u201352.","journal-title":"Am J Hematol"},{"key":"4147_CR8","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1007\/s12185-020-03008-3","volume":"113","author":"S Fujimoto","year":"2021","unstructured":"Fujimoto S, Kawabata H, Sakai T, Yanagisawa H, Nishikori M, Nara K, et al. Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan. Int J Hematol. 2021;113:73\u201380.","journal-title":"Int J Hematol"},{"key":"4147_CR9","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.2169\/internalmedicine.52.9482","volume":"52","author":"H Kawabata","year":"2013","unstructured":"Kawabata H, Kotani S, Matsumura Y, Kondo T, Katsurada T, Haga H, et al. Successful treatment of a patient with multicentric Castleman\u2019s disease who presented with thrombocytopenia, ascites, renal failure and myelofibrosis using tocilizumab, an anti-interleukin-6 receptor antibody. Intern Med. 2013;52:1503\u20137.","journal-title":"Intern Med"},{"key":"4147_CR10","doi-asserted-by":"publisher","first-page":"966","DOI":"10.1016\/S1470-2045(14)70319-5","volume":"15","author":"F van Rhee","year":"2014","unstructured":"van Rhee F, Wong RS, Munshi N, Rossi J-F, Ke X-Y, Foss\u00e5 A, et al. Siltuximab for multicentric Castleman\u2019s disease: a randomised, double-blind, placebo-controlled trial. Lancet Oncol. 2014;15:966\u201374.","journal-title":"Lancet Oncol"},{"key":"4147_CR11","doi-asserted-by":"publisher","first-page":"87","DOI":"10.3960\/jslrt.53.87","volume":"53","author":"N Iwaki","year":"2013","unstructured":"Iwaki N, Sato Y, Takata K, Kondo E, Ohno K, Takeuchi M, et al. Atypical hyaline vascular-type castleman\u2019s disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy. J Clin Exp Hematopathol. 2013;53:87\u201393.","journal-title":"J Clin Exp Hematopathol"},{"key":"4147_CR12","doi-asserted-by":"publisher","first-page":"95","DOI":"10.3960\/jslrt.53.95","volume":"53","author":"M Inoue","year":"2013","unstructured":"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\u202f: a case report. J Clin Exp Hematopathol. 2013;53:95\u20139.","journal-title":"J Clin Exp Hematopathol"},{"key":"4147_CR13","doi-asserted-by":"publisher","first-page":"799","DOI":"10.1056\/NEJMoa1110557","volume":"366","author":"S Verstovsek","year":"2012","unstructured":"Verstovsek S, Mesa RA, Gotlib J, Levy RS, Gupta V, DiPersio JF, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012;366:799\u2013807.","journal-title":"N Engl J Med"},{"key":"4147_CR14","doi-asserted-by":"publisher","first-page":"1800","DOI":"10.1056\/NEJMoa1917635","volume":"382","author":"R Zeiser","year":"2020","unstructured":"Zeiser R, von Bubnoff N, Butler J, Mohty M, Niederwieser D, Or R, et al. Ruxolitinib for glucocorticoid-refractory acute graft-versus-host disease. N Engl J Med. 2020;382:1800\u201310.","journal-title":"N Engl J Med"},{"key":"4147_CR15","doi-asserted-by":"publisher","first-page":"719","DOI":"10.7326\/L20-1051","volume":"174","author":"M Killian","year":"2021","unstructured":"Killian M, Viel S, Chalayer E, Forest F, Grange S, Bonnefoy P-B, et al. JAK1\/2 inhibition in severe TAFRO syndrome: a case report. Ann Intern Med. 2021;174:719\u201321.","journal-title":"Ann Intern Med"},{"key":"4147_CR16","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29261","volume":"68","author":"H Lust","year":"2021","unstructured":"Lust H, Gong S, Remiker A, Rossoff J. Idiopathic multicentric Castleman disease with TAFRO clinical subtype responsive to IL-6\/JAK inhibition: a pediatric case series. Pediatr Blood Cancer. 2021;68:e29261.","journal-title":"Pediatr Blood Cancer"},{"key":"4147_CR17","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000032200","volume":"101","author":"T Kakutani","year":"2022","unstructured":"Kakutani T, Nunokawa T, Chinen N, Tamai Y. Treatment-resistant idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly managed with Janus kinase inhibitors: a case report. Medicine (Baltimore). 2022;101:e32200.","journal-title":"Medicine (Baltimore)"},{"key":"4147_CR18","doi-asserted-by":"publisher","first-page":"61","DOI":"10.4274\/tjh.galenos.2024.2023.0477","volume":"41","author":"Y-H Gao","year":"2024","unstructured":"Gao Y-H, Duan M-H, Li J, Zhang L. Ruxolitinib for the treatment of refractory idiopathic multicentric Castleman disease: a case report. Turk J Hematol. 2024;41:61\u20133.","journal-title":"Turk J Hematol"},{"key":"4147_CR19","doi-asserted-by":"publisher","first-page":"11255","DOI":"10.3390\/cimb46100668","volume":"46","author":"T Kakutani","year":"2024","unstructured":"Kakutani T, Kamada R, Tamai Y. Pathophysiology, treatment, and prognosis of thrombocytopenia, anasarca, fever, reticulin fibrosis\/renal failure, and organomegaly (TAFRO) syndrome: a review. Curr Issues Mol Biol. 2024;46:11255\u201369.","journal-title":"Curr Issues Mol Biol"},{"key":"4147_CR20","doi-asserted-by":"publisher","first-page":"2391","DOI":"10.1182\/blood-2016-03-643544","volume":"127","author":"DA Arber","year":"2016","unstructured":"Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391\u2013405.","journal-title":"Blood"},{"key":"4147_CR21","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1007\/s12185-019-02780-1","volume":"111","author":"Y Masaki","year":"2020","unstructured":"Masaki Y, Kawabata H, Takai K, Tsukamoto N, Fujimoto S, Ishigaki Y, et al. 2019 updated diagnostic criteria and disease severity classification for TAFRO syndrome. Int J Hematol. 2020;111:155\u20138.","journal-title":"Int J Hematol"},{"key":"4147_CR22","doi-asserted-by":"publisher","first-page":"686","DOI":"10.1007\/s12185-016-1979-1","volume":"103","author":"Y Masaki","year":"2016","unstructured":"Masaki Y, Kawabata H, Takai K, Kojima M, Tsukamoto N, Ishigaki Y, et al. Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version. Int J Hematol. 2016;103:686\u201392.","journal-title":"Int J Hematol"},{"key":"4147_CR23","doi-asserted-by":"publisher","first-page":"2115","DOI":"10.1182\/blood-2018-07-862334","volume":"132","author":"F van Rhee","year":"2018","unstructured":"van Rhee F, Voorhees P, Dispenzieri A, Foss\u00e5 A, Srkalovic G, Ide M, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018;132:2115\u201324.","journal-title":"Blood"},{"key":"4147_CR24","doi-asserted-by":"publisher","first-page":"3445","DOI":"10.1182\/bloodadvances.2020004016","volume":"5","author":"SK Pierson","year":"2021","unstructured":"Pierson SK, Shenoy S, Oromendia AB, Gorzewski AM, Langan Pai R-A, Nabel CS, et al. Discovery and validation of a novel subgroup and therapeutic target in idiopathic multicentric Castleman disease. Blood Adv. 2021;5:3445\u201356.","journal-title":"Blood Adv"},{"key":"4147_CR25","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.135031","volume":"5","author":"R-AL Pai","year":"2020","unstructured":"Pai R-AL, Japp AS, Gonzalez M, Rasheed RF, Okumura M, Arenas D, et al. Type I IFN response associated with mTOR activation in the TAFRO subtype of idiopathic multicentric Castleman disease. JCI Insight. 2020;5:e135031.","journal-title":"JCI Insight"},{"key":"4147_CR26","volume":"2016","author":"M Simons","year":"2016","unstructured":"Simons M, Apor E, Butera JN, Treaba DO. TAFRO syndrome associated with EBV and successful triple therapy treatment: case report and review of the literature. Case Rep Hematol. 2016;2016:4703608.","journal-title":"Case Rep Hematol"},{"key":"4147_CR27","doi-asserted-by":"publisher","first-page":"660","DOI":"10.3324\/haematol.2015.141283","volume":"101","author":"AA Zahr","year":"2016","unstructured":"Zahr AA, Salama ME, Carreau N, Tremblay D, Verstovsek S, Mesa R, et al. Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies. Haematologica. 2016;101:660\u201371.","journal-title":"Haematologica"},{"key":"4147_CR28","doi-asserted-by":"publisher","DOI":"10.3390\/biom13010119","author":"J Liu","year":"2023","unstructured":"Liu J, Wang F, Luo F. The role of JAK\/STAT pathway in fibrotic diseases: molecular and cellular mechanisms. Biomolecules. 2023. https:\/\/doi.org\/10.3390\/biom13010119.","journal-title":"Biomolecules"},{"key":"4147_CR29","doi-asserted-by":"publisher","first-page":"1576","DOI":"10.1111\/cas.12812","volume":"106","author":"A Okamoto","year":"2015","unstructured":"Okamoto A, Yanada M, Miura H, Inaguma Y, Tokuda M, Morishima S, et al. Prognostic significance of Epstein-Barr virus DNA detection in pretreatment serum in diffuse large B-cell lymphoma. Cancer Sci. 2015;106:1576\u201381.","journal-title":"Cancer Sci"},{"key":"4147_CR30","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2021.710400","volume":"12","author":"Y Song","year":"2021","unstructured":"Song Y, Wang J, Wang Y, Wang Z. Ruxolitinib in patients with chronic active Epstein-Barr virus infection: a retrospective, single-center study. Front Pharmacol. 2021;12:710400.","journal-title":"Front Pharmacol"}],"container-title":["International Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12185-025-04147-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12185-025-04147-1","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12185-025-04147-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T11:56:32Z","timestamp":1776254192000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12185-025-04147-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,19]]},"references-count":30,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["4147"],"URL":"https:\/\/doi.org\/10.1007\/s12185-025-04147-1","relation":{},"ISSN":["0925-5710","1865-3774"],"issn-type":[{"value":"0925-5710","type":"print"},{"value":"1865-3774","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,19]]},"assertion":[{"value":"27 April 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 December 2025","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 December 2025","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 January 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare that they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interests"}},{"value":"The ethics committee of Sendai City Hospital approved this study. Informed consent was obtained from patients for publication of this article.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and informed consent"}}]}}