{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T21:48:11Z","timestamp":1790200091999,"version":"4.1.0"},"reference-count":31,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2022,9,7]],"date-time":"2022-09-07T00:00:00Z","timestamp":1662508800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"JST","award":["JPMJFS2128"],"award-info":[{"award-number":["JPMJFS2128"]}]},{"name":"JST","award":["JP 20K07407"],"award-info":[{"award-number":["JP 20K07407"]}]},{"name":"Japan Society for the Promotion of Science","award":["JPMJFS2128"],"award-info":[{"award-number":["JPMJFS2128"]}]},{"name":"Japan Society for the Promotion of Science","award":["JP 20K07407"],"award-info":[{"award-number":["JP 20K07407"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Idiopathic multicentric Castleman disease (iMCD) is a type of Castleman disease that is not related to KSHV\/HHV8 infection. Currently, iMCD is classified into iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) and iMCD-NOS (not otherwise specified). The former has been established as a relatively homogeneous disease unit that has been recently re-defined, while the latter is considered to be a heterogeneous disease that could be further divided into several subtypes. In 1980, Mori et al. proposed the concept of idiopathic plasmacytic lymphadenopathy (IPL), a disease presenting with polyclonal hypergammaglobulinemia and a sheet-like proliferation of mature plasma cells in the lymph nodes. Some researchers consider IPL to be a part of iMCD-NOS, although it has not been clearly defined to date. This is the first paper to analyze iMCD-NOS clinicopathologically, to examine whether IPL forms a uniform disease unit in iMCD. Histologically, the IPL group showed prominent plasmacytosis and the hyperplasia of germinal centers, while the non-IPL group showed prominent vascularity. Clinically, the IPL group showed significant thrombocytosis and elevated serum IgG levels compared to the non-IPL group (p = 0.007, p &lt; 0.001, respectively). Pleural effusion and ascites were less common in the IPL group (p &lt; 0.001). The IPL group was more likely to have an indolent clinical course and a good response to the anti-IL-6 receptor antibody, while the non-IPL counterpart frequently required more aggressive medical interventions. Thus, the IPL group is a clinicopathologically uniform entity that forms an independent subtype of iMCD.<\/jats:p>","DOI":"10.3390\/ijms231810301","type":"journal-article","created":{"date-parts":[[2022,9,7]],"date-time":"2022-09-07T20:52:03Z","timestamp":1662583923000},"page":"10301","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":53,"title":["Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1485-0527","authenticated-orcid":false,"given":"Asami","family":"Nishikori","sequence":"first","affiliation":[{"name":"Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama 700-8558, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8433-4870","authenticated-orcid":false,"given":"Midori Filiz","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama 700-8558, Japan"},{"name":"Department of Pathology, Okayama University Hospital, Okayama 700-8558, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0224-7501","authenticated-orcid":false,"given":"Yoshito","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan"},{"name":"Department of Medicine, John A. Burns School of Medicine, University of Hawai\u2019i, Honolulu, HI 96813, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7014-9095","authenticated-orcid":false,"given":"Fumio","family":"Otsuka","sequence":"additional","affiliation":[{"name":"Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kanna","family":"Maehama","sequence":"additional","affiliation":[{"name":"Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama 700-8558, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kumiko","family":"Ohsawa","sequence":"additional","affiliation":[{"name":"Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama 350-8550, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuji","family":"Momose","sequence":"additional","affiliation":[{"name":"Department of Pathology, Saitama Medical Center, Saitama Medical University, Saitama 350-8550, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4332-5254","authenticated-orcid":false,"given":"Naoya","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Pathology, Tokai University School of Medicine, Kanagawa 259-1193, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5234-6861","authenticated-orcid":false,"given":"Yasuharu","family":"Sato","sequence":"additional","affiliation":[{"name":"Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama 700-8558, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,9,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1002\/1097-0142(195607\/08)9:4<822::AID-CNCR2820090430>3.0.CO;2-4","article-title":"Localized mediastinal lymphnode hyperplasia resembling thymoma","volume":"9","author":"Castleman","year":"1956","journal-title":"Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Nishimura, M.F., Nishimura, Y., Nishikori, A., Maekawa, Y., Maehama, K., Yoshino, T., and Sato, Y. 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