{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T18:21:47Z","timestamp":1772994107479,"version":"3.50.1"},"reference-count":37,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T00:00:00Z","timestamp":1762128000000},"content-version":"vor","delay-in-days":306,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100017559","name":"Recordati Rare Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100017559","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Hematology"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Idiopathic multicentric Castleman disease (iMCD) is a rare lymphadenopathic disorder characterized by hyperplasia of multiple lymph nodes and can be associated with a wide range of symptoms and presentations, from mild disease to life\u2010threatening organ failure. Varied histopathological features and heterogeneous presentation of this rare entity can make the diagnosis quite challenging for both hematologists and other specialists who may encounter patients at various stages of disease progression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method<\/jats:title>\n                    <jats:p>We analyze five different clinical presentations at our institution to demonstrate challenging routes of diagnosis and treatment complexities of iMCD. We aim to raise awareness to the importance of early diagnosis and appropriate management of this rare condition.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>All patients in this series presented with symptomatic lymphadenopathy. We highlight one rare instance of thrombocytopenia, anasarca\/ascites, fever, reticulin fibrosis or renal dysfunction, and organomegaly (TAFRO) syndrome with elusive iMCD, which illustrates the challenges in the diagnosis of this rare condition and the importance of early recognition of its symptoms to avoid decompensation of patients. We also review established treatment guidelines and response criteria to siltuximab as outlined in the international consensus treatment guidelines.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>These cases highlight the heterogeneity and challenging diagnosis of this rare cytokine\u2010driven hematological disorder and the role of siltuximab in the treatment of iMCD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1155\/crh\/3377688","type":"journal-article","created":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T13:55:33Z","timestamp":1762178133000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Heterogeneous Presentations of iMCD: A Single\u2010Institution Case Series"],"prefix":"10.1155","volume":"2025","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4125-5355","authenticated-orcid":false,"given":"Jaspreet","family":"Kaur","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7262-3985","authenticated-orcid":false,"given":"Anthony","family":"Sisk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9758-2147","authenticated-orcid":false,"given":"Jonathan E.","family":"Zuckerman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3549-0981","authenticated-orcid":false,"given":"Haifaa","family":"Abdulhaq","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,11,3]]},"reference":[{"key":"e_1_2_16_1_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000931"},{"key":"e_1_2_16_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-022-04762-6"},{"key":"e_1_2_16_3_2","first-page":"320","article-title":"Thrombocytopenia With Mild Bone Marrow Fibrosis Accompanied by Fever, Pleural Effusion, Ascites and Hepatosplenomegaly","volume":"51","author":"Takai K.","year":"2010","journal-title":"Rinsho Ketsueki"},{"key":"e_1_2_16_4_2","doi-asserted-by":"publisher","DOI":"10.3960\/jslrt.53.57"},{"key":"e_1_2_16_5_2","unstructured":"Castleman Disease Collaborative Network First-Ever Diagnostic Criteria and ICD-10 Code 2016 https:\/\/cdcn.org\/news\/first-ever-diagnostic-criteria-and-icd-10-code-for-castleman-disease\/."},{"key":"e_1_2_16_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-10-746933"},{"key":"e_1_2_16_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(14)70319-5"},{"key":"e_1_2_16_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S2352-3026(19)30257-1"},{"key":"e_1_2_16_9_2","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2022007112"},{"key":"e_1_2_16_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2017.09.002"},{"key":"e_1_2_16_11_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0218660"},{"key":"e_1_2_16_12_2","doi-asserted-by":"publisher","DOI":"10.1159\/000518079"},{"key":"e_1_2_16_13_2","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2021004441"},{"key":"e_1_2_16_14_2","unstructured":"Referenced With Permission From the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae) for Castleman Disease V.2.2025. \u00a9 National Comprehensive Cancer Network Inc. 2025. All Rights Reserved. Accessed September 2025. To View the Most Recent and Complete Version of the Guideline Go to NCCN.org. NCCN Makes No Warranties of Any Kind Whatsoever Regarding Their Content Use or Application and Disclaims Any Responsibility for Their Application or Use in Any Way."},{"key":"e_1_2_16_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26292"},{"key":"e_1_2_16_16_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-07-862334"},{"key":"e_1_2_16_17_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-03-278127"},{"key":"e_1_2_16_18_2","doi-asserted-by":"publisher","DOI":"10.3109\/10428194.2014.953145"},{"key":"e_1_2_16_19_2","doi-asserted-by":"publisher","DOI":"10.2147\/JBM.S127822"},{"key":"e_1_2_16_20_2","doi-asserted-by":"publisher","DOI":"10.3960\/jslrt.53.79"},{"key":"e_1_2_16_21_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2008.08.026"},{"key":"e_1_2_16_22_2","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfr245"},{"key":"e_1_2_16_23_2","doi-asserted-by":"publisher","DOI":"10.1097\/RHU.0000000000001810"},{"key":"e_1_2_16_24_2","doi-asserted-by":"publisher","DOI":"10.1007\/s13730-020-00559-6"},{"key":"e_1_2_16_25_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12882-017-0472-2"},{"key":"e_1_2_16_26_2","doi-asserted-by":"publisher","DOI":"10.1007\/s13730-020-00511-8"},{"key":"e_1_2_16_27_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12882-020-02191-z"},{"key":"e_1_2_16_28_2","doi-asserted-by":"publisher","DOI":"10.2147\/PROM.S140011"},{"key":"e_1_2_16_29_2","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-15-0134"},{"key":"e_1_2_16_30_2","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.15588"},{"key":"e_1_2_16_31_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41388-020-1204-9"},{"key":"e_1_2_16_32_2","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.13702"},{"key":"e_1_2_16_33_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2023021992"},{"key":"e_1_2_16_34_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27132"},{"key":"e_1_2_16_35_2","doi-asserted-by":"publisher","DOI":"10.1002\/ccr3.2502"},{"key":"e_1_2_16_36_2","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2022.974770"},{"key":"e_1_2_16_37_2","doi-asserted-by":"publisher","DOI":"10.4274\/tjh.galenos.2024.2024.0339"}],"container-title":["Case Reports in Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crh\/3377688","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1155\/crh\/3377688","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crh\/3377688","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T15:04:48Z","timestamp":1772982288000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1155\/crh\/3377688"}},"subtitle":[],"editor":[{"given":"Kalyani","family":"Sen","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":37,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1155\/crh\/3377688"],"URL":"https:\/\/doi.org\/10.1155\/crh\/3377688","archive":["Portico"],"relation":{},"ISSN":["2090-6560","2090-6579"],"issn-type":[{"value":"2090-6560","type":"print"},{"value":"2090-6579","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1]]},"assertion":[{"value":"2024-06-14","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-10-11","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-03","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"3377688"}}