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Multicentric CD (MCD) is further subdivided into human herpesvirus type\u20108\u2010associated, POEMS syndrome\u2010associated, and idiopathic (iMCD). TAFRO syndrome is a newly identified disorder of unknown etiology characterized by\n                    <jats:bold>t<\/jats:bold>\n                    hrombocytopenia,\n                    <jats:bold>a<\/jats:bold>\n                    nasarca,\n                    <jats:bold>f<\/jats:bold>\n                    ever,\n                    <jats:bold>r<\/jats:bold>\n                    eticulin myelofibrosis, renal dysfunction, and\n                    <jats:bold>o<\/jats:bold>\n                    rganomegaly. The TAFRO syndrome is sometimes regarded as a subtype of iMCD (TAFRO\u2010iMCD), whereas iMCD without TAFRO syndrome is considered \u201cnot otherwise specified\u201d (iMCD\u2010NOS). However, a proportion of patients with TAFRO syndrome have been diagnosed without lymph node biopsies (TAFRO syndrome without proven iMCD; TAFRO\u2010w\/op\u2010iMCD). To clarify the clinical features of iMCD\u2010NOS, TAFRO\u2010iMCD, and TAFRO\u2010w\/op\u2010iMCD, we retrospectively analyzed 220 patients extracted from the database of the Multicenter Collaborative Retrospective Study for Establishing the Concept of TAFRO Syndrome. The patients included 87 with iMCD\u2010NOS, 63 with TAFRO\u2010iMCD, and 19 with TAFRO\u2010w\/op\u2010iMCD. Patients in all three groups exhibited anemia, hypoalbuminemia, and elevated serum C\u2010reactive protein and interleukin\u20106 levels. No significant differences in clinical, laboratory, and prognostic features were noted between the TAFRO\u2010iMCD, and TAFRO\u2010w\/op\u2010iMCD groups. However, the iMCD\u2010NOS group exhibited polyclonal hyper\u2010\u03b3\u2010globulinemia. The five\u2010year survival rates of patients in the iMCD\u2010NOS and TAFRO\u2010involved groups were 100% and 66.5%, respectively (dropping markedly during the first few months in the latter). The iMCD\u2010NOS and the TAFRO\u2010iMCD samples typically showed plasma cell and mixed\u2010type histologies, respectively. Thus, iMCD can be classified into two distinct subtypes, iMCD\u2010NOS and TAFRO\u2010iMCD. As such, TAFRO\u2010iMCD and TAFRO\u2010w\/op\u2010iMCD may be considered the same entity, requiring prompt diagnosis and intensive care.\n                  <\/jats:p>","DOI":"10.1002\/ajh.25554","type":"journal-article","created":{"date-parts":[[2019,6,10]],"date-time":"2019-06-10T07:59:06Z","timestamp":1560153546000},"page":"975-983","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":99,"title":["Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?"],"prefix":"10.1002","volume":"94","author":[{"given":"Shino","family":"Fujimoto","sequence":"first","affiliation":[{"name":"Department of Hematology and Immunology Kanazawa Medical University  Uchinada Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomoyuki","family":"Sakai","sequence":"additional","affiliation":[{"name":"Department of Hematology and Immunology Kanazawa Medical University  Uchinada 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