{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T16:47:43Z","timestamp":1774284463779,"version":"3.50.1"},"reference-count":42,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T00:00:00Z","timestamp":1759968000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"JSPS KAKENHI","award":["JP 24K18671"],"award-info":[{"award-number":["JP 24K18671"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,3,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>This study aimed to evaluate treatment outcomes, complications, and the clinical course of thrombocytopenia in patients with TAFRO syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a retrospective analysis of patients treated at Nara Medical University Hospital from 2015 to 2023. Diagnosis of TAFRO syndrome was made according to the 2015 or 2019 criteria, and disease severity was assessed based on the updated disease severity classification. Clinical characteristics and laboratory and histological data were analysed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Seven patients were included, with four classified as very severe, two as severe, and one as slightly severe. All the patients received early combination therapy (various combinations of glucocorticoids, tocilizumab, cyclosporine, and rituximab), with the second therapy added a median of 5\u00a0days after the first. Six patients received eltrombopag. Despite intensive treatment, thrombocytopenia persisted for a prolonged period, with a median time to platelet count normalization of 54\u00a0days. Cytomegalovirus reactivation occurred in six patients during treatment but resolved with appropriate antiviral therapy. All the patients achieved remission and survived.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Thrombocytopenia in TAFRO syndrome persists long term despite intensive combination therapy. Early combination therapy was associated with excellent survival outcomes, suggesting that intensifying treatment solely for thrombocytopenia may not be necessary given its prolonged course.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/mr\/roaf092","type":"journal-article","created":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T12:08:11Z","timestamp":1759925291000},"page":"299-306","source":"Crossref","is-referenced-by-count":1,"title":["Tafro syndrome: early combination therapy outcomes and prolonged thrombocytopenia\u2014a seven-case series"],"prefix":"10.1093","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8956-2725","authenticated-orcid":false,"given":"Shiro","family":"Ono","sequence":"first","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomohiro","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5163-0481","authenticated-orcid":false,"given":"Nobushiro","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hidetoshi","family":"Matsuoka","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0382-7498","authenticated-orcid":false,"given":"Ryo","family":"Yoneima","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9345-0665","authenticated-orcid":false,"given":"Hiromasa","family":"Kawashima","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makiko","family":"Miyamoto","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6337-3280","authenticated-orcid":false,"given":"Masaki","family":"Matsubara","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8434-7399","authenticated-orcid":false,"given":"Noritaka","family":"Yada","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5823-8239","authenticated-orcid":false,"given":"Kiyomi","family":"Yoshimoto","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Nara Medical University , Nara ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,10,9]]},"reference":[{"key":"2026030508163864700_ref1","first-page":"320","article-title":"Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly","volume":"51","author":"Takai","year":"2010","journal-title":"Rinsho Ketsueki"},{"key":"2026030508163864700_ref2","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1002\/ajh.24242","article-title":"Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease","volume":"91","author":"Iwaki","year":"2016","journal-title":"Am J Hematol"},{"key":"2026030508163864700_ref3","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1002\/ajh.26292","article-title":"Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease","volume":"96","author":"Nishimura","year":"2021","journal-title":"Am J Hematol"},{"key":"2026030508163864700_ref4","doi-asserted-by":"crossref","first-page":"27","DOI":"10.2169\/internalmedicine.9622-22","article-title":"TAFRO syndrome: a disease requiring immediate medical attention","volume":"62","author":"Masaki","year":"2023","journal-title":"Intern Med Tokyo Jpn"},{"key":"2026030508163864700_ref5","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1002\/ajh.25554","article-title":"Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?","volume":"94","author":"Fujimoto","year":"2019","journal-title":"Am J Hematol"},{"key":"2026030508163864700_ref6","doi-asserted-by":"crossref","first-page":"e9220","DOI":"10.1097\/MD.0000000000009220","article-title":"Sj\u00f6gren\u2019s syndrome manifesting as clinicopathological features of TAFRO syndrome: a case report","volume":"96","author":"Fujimoto","year":"2017","journal-title":"Medicine (Baltimore)"},{"key":"2026030508163864700_ref7","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1177\/0961203317725589","article-title":"Characterization of patients with systemic lupus erythematosus who meet the diagnostic criteria for TAFRO syndrome","volume":"27","author":"Hasegawa","year":"2018","journal-title":"Lupus"},{"key":"2026030508163864700_ref8","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1007\/s12185-020-03008-3","article-title":"Optimal treatments for TAFRO syndrome: retrospective surveillance study in Japan","volume":"113","author":"Fujimoto","year":"2021","journal-title":"Int J Hematol"},{"key":"2026030508163864700_ref9","doi-asserted-by":"crossref","first-page":"e135031","DOI":"10.1172\/jci.insight.135031","article-title":"Type I IFN response associated with mTOR activation in the TAFRO subtype of idiopathic multicentric Castleman 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lesions","volume":"62","author":"Nakayama","year":"2023","journal-title":"Intern Med Tokyo Jpn"},{"key":"2026030508163864700_ref16","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1007\/s12185-019-02604-2","article-title":"Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab","volume":"109","author":"Suzuki","year":"2019","journal-title":"Int J Hematol"},{"key":"2026030508163864700_ref17","doi-asserted-by":"crossref","first-page":"905.e1","DOI":"10.1016\/j.crad.2017.05.007","article-title":"CT findings in 11 patients with TAFRO syndrome: a variant of multicentric Castleman\u2019s disease","volume":"72","author":"Kiguchi","year":"2017","journal-title":"Clin Radiol"},{"key":"2026030508163864700_ref18","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1016\/j.resinv.2016.10.003","article-title":"Two patients with TAFRO syndrome exhibiting strikingly similar anterior mediastinal lesions with predominantly fat attenuation on chest computed tomography","volume":"55","author":"Ozawa","year":"2017","journal-title":"Respir Investig"},{"key":"2026030508163864700_ref19","doi-asserted-by":"crossref","first-page":"1303","DOI":"10.2169\/internalmedicine.5850-20","article-title":"Complete resolution of a case of TAFRO syndrome accompanied by mediastinal panniculitis, adrenal lesion, and liver damage with hyperbilirubinemia","volume":"60","author":"Ono","year":"2021","journal-title":"Intern Med Tokyo Jpn"},{"key":"2026030508163864700_ref20","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1007\/s10554-013-0306-7","article-title":"Anterior mediastinal fat in Beh\u00e7et\u2019s disease: qualitative and quantitative CT analysis","volume":"29","author":"Lee","year":"2013","journal-title":"Int J Cardiovasc 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Int"},{"key":"2026030508163864700_ref24","doi-asserted-by":"crossref","first-page":"2388","DOI":"10.1002\/ccr3.2502","article-title":"Multicentric Castleman disease with TAFRO syndrome and Sj\u00f6gren\u2019s","volume":"7","author":"Li","year":"2019","journal-title":"Clin Case Rep"},{"key":"2026030508163864700_ref25","first-page":"8872774","article-title":"Primary Sj\u00f6gren\u2019s syndrome accompanied by clinical features of TAFRO syndrome","volume":"2020","author":"Suzuki","year":"2020","journal-title":"Case Rep Rheumatol"},{"key":"2026030508163864700_ref26","doi-asserted-by":"crossref","first-page":"103137","DOI":"10.1016\/j.autrev.2022.103137","article-title":"TAFRO syndrome: a severe manifestation of Sjogren\u2019s syndrome? A systematic review","volume":"21","author":"Grange","year":"2022","journal-title":"Autoimmun Rev"},{"key":"2026030508163864700_ref27","doi-asserted-by":"crossref","first-page":"2889","DOI":"10.1038\/s41598-024-53413-5","article-title":"TAFRO syndrome is associated with anti-SSA\/Ro60 antibodies, in contrast to idiopathic castleman disease","volume":"14","author":"Shirakashi","year":"2024","journal-title":"Sci Rep"},{"key":"2026030508163864700_ref28","doi-asserted-by":"crossref","first-page":"3515","DOI":"10.1007\/s00277-023-05435-8","article-title":"The experience of diagnosis and treatment for TAFRO syndrome","volume":"102","author":"Wu","year":"2023","journal-title":"Ann Hematol"},{"key":"2026030508163864700_ref29","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.3390\/biomedicines12061277","article-title":"TAFRO syndrome: guidance for managing patients presenting thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly","volume":"12","author":"Miura","year":"2024","journal-title":"Biomedicines"},{"key":"2026030508163864700_ref30","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1080\/24725625.2020.1717747","article-title":"Recovery from prolonged thrombocytopenia in patients with TAFRO syndrome: case series and literature review","volume":"4","author":"Yamaguchi","year":"2020","journal-title":"Mod Rheumatol Case Rep"},{"key":"2026030508163864700_ref31","doi-asserted-by":"crossref","first-page":"2715","DOI":"10.2169\/internalmedicine.1178-22","article-title":"Severe TAFRO syndrome mimicking hepatorenal syndrome successfully treated with a multidisciplinary approach: a case report and literature review","volume":"62","author":"Yamamoto","year":"2023","journal-title":"Intern Med Tokyo Jpn"},{"key":"2026030508163864700_ref32","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.2169\/internalmedicine.52.9482","article-title":"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","volume":"52","author":"Kawabata","year":"2013","journal-title":"Intern Med Tokyo Jpn"},{"key":"2026030508163864700_ref33","doi-asserted-by":"crossref","first-page":"95","DOI":"10.3960\/jslrt.53.95","article-title":"Complete resolution of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) after immunosuppressive therapies using corticosteroids and cyclosporin A: a case report","volume":"53","author":"Inoue","year":"2013","journal-title":"J Clin Exp Hematop"},{"key":"2026030508163864700_ref34","doi-asserted-by":"crossref","first-page":"87","DOI":"10.3960\/jslrt.53.87","article-title":"Atypical hyaline vascular-type castleman\u2019s disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy","volume":"53","author":"Iwaki","year":"2013","journal-title":"J Clin Exp Hematop"},{"key":"2026030508163864700_ref35","doi-asserted-by":"crossref","first-page":"79","DOI":"10.3960\/jslrt.53.79","article-title":"Japanese variant of multicentric castleman\u2019s disease associated with serositis and thrombocytopenia--a report of two cases: is TAFRO syndrome (Castleman-Kojima disease) a distinct clinicopathological entity?","volume":"53","author":"Masaki","year":"2013","journal-title":"J Clin Exp Hematop"},{"key":"2026030508163864700_ref36","doi-asserted-by":"crossref","first-page":"1291","DOI":"10.2169\/internalmedicine.9484-17","article-title":"TAFRO syndrome with disseminated intravascular coagulation successfully treated with tocilizumab and recombinant thrombomodulin","volume":"57","author":"Takayama","year":"2018","journal-title":"Intern Med Tokyo Jpn"},{"key":"2026030508163864700_ref37","doi-asserted-by":"crossref","first-page":"6286","DOI":"10.3390\/ijms22126286","article-title":"TAFRO syndrome with renal thrombotic microangiopathy: insights into the molecular mechanism and treatment opportunities","volume":"22","author":"Tu","year":"2021","journal-title":"Int J Mol Sci"},{"key":"2026030508163864700_ref38","doi-asserted-by":"crossref","first-page":"3829","DOI":"10.1182\/bloodadvances.2019000966","article-title":"American Society of Hematology 2019 guidelines for immune thrombocytopenia","volume":"3","author":"Neunert","year":"2019","journal-title":"Blood Adv"},{"key":"2026030508163864700_ref39","doi-asserted-by":"crossref","first-page":"1489","DOI":"10.3389\/fimmu.2019.01489","article-title":"Renal pathologic findings in TAFRO syndrome: is there a continuum between thrombotic microangiopathy and membranoproliferative glomerulonephritis? A case report and literature review","volume":"10","author":"Leurs","year":"2019","journal-title":"Front Immunol"},{"key":"2026030508163864700_ref40","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1186\/s12882-019-1574-9","article-title":"TAFRO syndrome as a cause of glomerular microangiopathy: a case report and literature review","volume":"20","author":"Nagayama","year":"2019","journal-title":"BMC Nephrol"},{"key":"2026030508163864700_ref41","doi-asserted-by":"crossref","first-page":"e234155","DOI":"10.1136\/bcr-2019-234155","article-title":"Unexplained cause of thrombocytopenia, fever, anasarca and hypothyroidism: TAFRO syndrome with thrombotic microangiopathy renal histology","volume":"13","author":"Simeni Njonnou","year":"2020","journal-title":"BMJ Case Rep"},{"key":"2026030508163864700_ref42","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s10157-024-02573-9","article-title":"Renal involvement in TAFRO syndrome: a review","volume":"29","author":"Ubara","year":"2025","journal-title":"Clin Exp Nephrol"}],"container-title":["Modern Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/mr\/advance-article-pdf\/doi\/10.1093\/mr\/roaf092\/64573408\/roaf092.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mr\/article-pdf\/36\/2\/299\/64573408\/roaf092.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mr\/article-pdf\/36\/2\/299\/64573408\/roaf092.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T13:16:48Z","timestamp":1772716608000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/mr\/article\/36\/2\/299\/8279880"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,9]]},"references-count":42,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2025,10,9]]},"published-print":{"date-parts":[[2026,3,5]]}},"URL":"https:\/\/doi.org\/10.1093\/mr\/roaf092","relation":{},"ISSN":["1439-7595","1439-7609"],"issn-type":[{"value":"1439-7595","type":"print"},{"value":"1439-7609","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,3]]},"published":{"date-parts":[[2025,10,9]]}}}