{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T08:03:47Z","timestamp":1780041827048,"version":"3.53.1"},"reference-count":32,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T00:00:00Z","timestamp":1779753600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Oncol Pharm Pract"],"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To review current pharmacologic strategies for Castleman disease (CD), integrating contemporary understanding of disease biology, diagnostic subclassification, and therapeutic approaches across unicentric Castleman disease (UCD); Human Herpesvirus-8 (HHV-8)\u2013associated multicentric Castleman disease (MCD); polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-cell disorder, skin changes (POEMS)\u2013associated MCD; and idiopathic MCD (iMCD).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Data Sources<\/jats:title>\n                    <jats:p>A comprehensive search of PubMed, Embase, and the Cochrane Library (2000\u20132025) was performed, supplemented by manual review of references and guidelines from the Castleman Disease Collaborative Network (CDCN), the National Comprehensive Cancer Network (NCCN), and the American Society of Clinical Oncology (ASCO). Included literature encompassed clinical trials, natural-history studies, and mechanistic work relevant to CD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Data Summary<\/jats:title>\n                    <jats:p>CD represents a biologically heterogeneous spectrum driven largely by dysregulated cytokine signaling, particularly interleukin-6 (IL-6). UCD is typically approached with surgical excision, whereas MCD requires subtype-specific systemic therapy. Rituximab remains the cornerstone of HHV-8\u2013associated MCD. Management of POEMS-associated disease focuses on controlling the plasma-cell clone. In iMCD, IL-6\u2013directed therapies (siltuximab or tocilizumab) represent first-line treatment, often supported by corticosteroids. Subtypes differ biologically: iMCD-idiopathic plasmacytic lymphadenopathy (iMCD-IPL) generally responds well to IL-6 blockade, whereas iMCD with thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (iMCD-TAFRO) is aggressive and may require early escalation to cyclosporine, rituximab, or chemotherapy. Emerging data showing mTORC1 hyperactivation provide rationale for mTOR inhibition, while case-level responses to JAK-2 inhibitors warrant further study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>CD is clinically and biologically diverse. IL-6 blockade, B-cell-directed therapy, and plasma-cell-targeted strategies anchor current management, while mTOR and JAK-2 inhibitors merit investigation in the relapsed\/refractory setting.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/10781552261455763","type":"journal-article","created":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T12:36:54Z","timestamp":1779799014000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Updates in the pharmacologic management of various Castleman disease subtypes: A comprehensive review"],"prefix":"10.1177","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3445-7546","authenticated-orcid":false,"given":"Victor M","family":"Samperio","sequence":"first","affiliation":[{"name":"Eisenhower Health"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sujay","family":"Singh","sequence":"additional","affiliation":[{"name":"Eisenhower Health"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahum","family":"Zahid","sequence":"additional","affiliation":[{"name":"Eisenhower Health"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0425-8394","authenticated-orcid":false,"given":"Constantin A","family":"Dasanu","sequence":"additional","affiliation":[{"name":"Christus Health"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,5,26]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-07-862334"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2021004441"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.5603\/AHP.2021.0005"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.5152\/TurkThoracJ.2018.18066"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000931"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2024013548"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/cas.13439"},{"key":"e_1_3_2_9_2","first-page":"2196","article-title":"Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease","volume":"109","author":"Bustamante MS","year":"2024","unstructured":"Bustamante MS, Pierson SK, Ren Y, et al. Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease. Haematologica 2024; 109: 2196\u20132206.","journal-title":"Haematologica"},{"key":"e_1_3_2_10_2","first-page":"564","article-title":"How we manage idiopathic multicentric Castleman disease","volume":"20","author":"Brandstadter JD","year":"2022","unstructured":"Brandstadter JD, Fajgenbaum DC. How we manage idiopathic multicentric Castleman disease. Clin Adv Hematol Oncol 2022; 20: 564\u2013571.","journal-title":"Clin Adv Hematol Oncol"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S2352-3026(16)00006-5"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.3390\/ijms231810301"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2020003334"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-10-747477"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25495"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25100"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-019-0391-2"},{"key":"e_1_3_2_18_2","first-page":"888","article-title":"What is the best treatment strategy before autologous peripheral blood stem cell transplantation in POEMS syndrome?","volume":"109","author":"Autore F","year":"2024","unstructured":"Autore F, Bramanti S, Lessi F, et al. What is the best treatment strategy before autologous peripheral blood stem cell transplantation in POEMS syndrome? Haematologica 2024; 109: 888\u2013894.","journal-title":"Haematologica"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-04-423178"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2023010745"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(14)70319-5"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1097\/HS9.0000000000000891"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.19334"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.3390\/biomedicines12061277"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25554"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.55.4710"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.10022"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019002792"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-024-05783-z"},{"key":"e_1_3_2_30_2","article-title":"Treatment-resistant idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly managed with Janus kinase inhibitors: a case report","volume":"101","author":"Kakutani T","unstructured":"Kakutani T, Nunokawa T, Chinen N, et al. 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 Dec 2; 101: e32200.","journal-title":"Medicine (Baltimore)"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.7326\/L20-1051"},{"key":"e_1_3_2_32_2","article-title":"Idiopathic multicentric castleman disease with TAFRO clinical subtype responsive to IL-6\/JAK inhibition: a pediatric case series","volume":"68","author":"Lust H","unstructured":"Lust H, Gong S, Remiker A, et al. Idiopathic multicentric castleman disease with TAFRO clinical subtype responsive to IL-6\/JAK inhibition: a pediatric case series. 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