{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T10:16:27Z","timestamp":1780049787120,"version":"3.53.1"},"reference-count":64,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2024,10,9]],"date-time":"2024-10-09T00:00:00Z","timestamp":1728432000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CIMB"],"abstract":"<jats:p>TAFRO syndrome, first reported in 2010, is a systemic inflammatory disease with a rapid onset and potentially fatal course if not treated promptly and appropriately. The name is derived from the initial letters describing the characteristic symptoms of thrombocytopenia, anasarca, fever, reticulin fibrosis\/renal failure, and organomegaly. It is sometimes considered a special subtype of idiopathic multicentric Castleman disease (iMCD) because lymph node biopsies often reveal the pathology findings seen in iMCD. However, its clinical manifestations and prognoses are not well documented. Since the clinical manifestations and prognoses of TAFRO syndrome differ significantly from those of iMCD, it is recognized as an independent disease concept and considered to partially overlap with the pathology of MCD. The pathogenesis of TAFRO syndrome remains largely unknown. Due to the lack of appropriate treatment, it often presents with multiple organ dysfunction and fatality. In this review, we summarized new findings on the pathogenesis of TAFRO syndrome and discussed current effective therapies and future treatment strategies.<\/jats:p>","DOI":"10.3390\/cimb46100668","type":"journal-article","created":{"date-parts":[[2024,10,10]],"date-time":"2024-10-10T08:16:42Z","timestamp":1728548202000},"page":"11255-11269","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Pathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis\/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review"],"prefix":"10.3390","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7760-8581","authenticated-orcid":false,"given":"Takuya","family":"Kakutani","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Shonan Kamakura General Hospital, Kanagawa 247-8533, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riko","family":"Kamada","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Shonan Kamakura General Hospital, Kanagawa 247-8533, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4013-1737","authenticated-orcid":false,"given":"Yotaro","family":"Tamai","sequence":"additional","affiliation":[{"name":"Division of Hematology, Shonan Kamakura General Hospital, Kanagawa 247-8533, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,10,9]]},"reference":[{"key":"ref_1","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":"ref_2","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. 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