{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T13:10:29Z","timestamp":1789564229310,"version":"build-2803163510"},"reference-count":62,"publisher":"Harborside Press, LLC","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:p>Castleman disease (CD) comprises a heterogeneous group of rare lymphoproliferative disorders unified by characteristic lymph node histopathology but with substantial clinical, biologic, and therapeutic diversity. Over the past decade, major advances in disease classification, pathophysiologic understanding, and targeted therapy have reshaped the management of CD, particularly idiopathic multicentric CD (iMCD). The introduction of IL-6\u2013directed therapy has transformed the management of iMCD by shifting treatment from empirical immunosuppression or cytotoxic chemotherapy toward a biologic-based approach, achieving durable disease control in most patients. Progress has also been made over the past several years in the anatomic, etiologic, and clinical classification of CD, enabling more individualized, severity-adapted treatment approaches across the disease spectrum. Emerging paradigms in CD management include the recognition of oligocentric CD (OligoCD) as an intermediate anatomic entity with distinct therapeutic implications, as well as the validation of idiopathic plasmacytic lymphadenopathy (IPL) as a clinically distinct subtype of iMCD. These developments underscore the importance of integrating disease distribution, subtype, and severity into therapeutic decision-making. In addition, the recent paradigm of CD management emphasizes early identification of refractory disease and timely escalation beyond biologic therapy. This review provides an updated overview of CD management, drawing on recent advances to discuss practical considerations in treatment selection across disease subtypes, as well as ongoing challenges and future directions.<\/jats:p>","DOI":"10.6004\/jnccn.2026.7046","type":"journal-article","created":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T12:44:39Z","timestamp":1788266679000},"source":"Crossref","is-referenced-by-count":0,"title":["New Paradigms in the Management of Castleman Disease"],"prefix":"10.6004","volume":"24","author":[{"given":"Yoshito","family":"Nishimura","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas M.","family":"Habermann","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariela","family":"Noy","sequence":"additional","affiliation":[{"name":"Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY"},{"name":"Weill Cornell Medical College, New York, NY"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"4047","reference":[{"key":"B1","series-title":"Cancer","first-page":"822","article-title":"Localized mediastinal lymph node hyperplasia resembling thymoma","volume":"9","author":"Castleman B","year":"1956","unstructured":"Castleman B, Iverson L, Menendez VP. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 1956;9:822\u2013830."},{"key":"B2","series-title":"Am J Hematol","first-page":"997","article-title":"The clinical spectrum of Castleman's disease","volume":"87","author":"Dispenzieri A","year":"2012","unstructured":"Dispenzieri A, Armitage JO, Loe MJ,  The clinical spectrum of Castleman's disease. Am J Hematol 2012;87:997\u20131002."},{"key":"B3","series-title":"Blood","first-page":"2924","article-title":"HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy","volume":"123","author":"Fajgenbaum DC","year":"2014","unstructured":"Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy. Blood 2014;123:2924\u20132933."},{"key":"B4","series-title":"Blood","first-page":"1646","article-title":"International, evidence-based consensus diagnostic criteria for HHV-8-negative\/idiopathic multicentric Castleman disease","volume":"129","author":"Fajgenbaum DC","year":"2017","unstructured":"Fajgenbaum DC, Uldrick TS, Bagg A,  International, evidence-based consensus diagnostic criteria for HHV-8-negative\/idiopathic multicentric Castleman disease. Blood 2017;129:1646\u20131657."},{"key":"B5","series-title":"Blood","first-page":"2627","article-title":"Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease","volume":"106","author":"Nishimoto N","year":"2005","unstructured":"Nishimoto N, Kanakura Y, Aozasa K,  Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood 2005;106:2627\u20132632."},{"key":"B6","series-title":"J Clin Oncol","first-page":"3701","article-title":"Siltuximab, a novel anti-interleukin-6 monoclonal antibody, for Castleman's disease","volume":"28","author":"Rhee F","year":"2010","unstructured":"Rhee F, Fayad L, Voorhees P,  Siltuximab, a novel anti-interleukin-6 monoclonal antibody, for Castleman's disease. J Clin Oncol 2010;28:3701\u20133708."},{"key":"B7","series-title":"Blood","first-page":"4189","article-title":"Human and viral interleukin-6 and other cytokines in Kaposi sarcoma herpesvirus-associated multicentric Castleman disease","volume":"122","author":"Polizzotto MN","year":"2013","unstructured":"Polizzotto MN, Uldrick TS, Wang V,  Human and viral interleukin-6 and other cytokines in Kaposi sarcoma herpesvirus-associated multicentric Castleman disease. Blood 2013;122:4189\u20134198."},{"key":"B8","series-title":"Clin Cancer Res","first-page":"1248","article-title":"Interleukin-6 as a therapeutic target","volume":"21","author":"Rossi JF","year":"2015","unstructured":"Rossi JF, Lu ZY, Jourdan M, Klein B. Interleukin-6 as a therapeutic target. Clin Cancer Res 2015;21:1248\u20131257."},{"key":"B9","series-title":"Ann Intern Med","first-page":"836","article-title":"Brief communication: rituximab in HIV-associated multicentric Castleman disease","volume":"147","author":"Bower M","year":"2007","unstructured":"Bower M, Powles T, Williams S,  Brief communication: rituximab in HIV-associated multicentric Castleman disease. Ann Intern Med 2007;147:836\u2013839."},{"key":"B10","series-title":"J Clin Oncol","first-page":"3350","article-title":"Prospective study of rituximab in chemotherapy-dependent human immunodeficiency virus associated multicentric Castleman's disease: ANRS 117 CastlemaB trial","volume":"25","author":"G\u00e9rard L","year":"2007","unstructured":"G\u00e9rard L, B\u00e9rezn\u00e9 A, Galicier L,  Prospective study of rituximab in chemotherapy-dependent human immunodeficiency virus associated multicentric Castleman's disease: ANRS 117 CastlemaB trial. J Clin Oncol 2007;25:3350\u20133356."},{"key":"B11","series-title":"Lancet Haematol","first-page":"e209","article-title":"Long-term safety of siltuximab in patients with idiopathic multicentric Castleman disease: a prespecified, open-label, extension analysis of two trials","volume":"7","author":"van Rhee F","year":"2020","unstructured":"van Rhee F, Casper C, Voorhees PM,  Long-term safety of siltuximab in patients with idiopathic multicentric Castleman disease: a prespecified, open-label, extension analysis of two trials. Lancet Haematol 2020;7:e209\u2013217."},{"key":"B12","series-title":"Blood Adv","first-page":"4773","article-title":"Siltuximab is associated with improved progression-free survival in idiopathic multicentric Castleman disease","volume":"6","author":"van Rhee F","year":"2022","unstructured":"van Rhee F, Rosenthal A, Kanhai K,  Siltuximab is associated with improved progression-free survival in idiopathic multicentric Castleman disease. Blood Adv 2022;6:4773\u20134781."},{"key":"B13","series-title":"Lancet Oncol","first-page":"966","article-title":"Siltuximab for multicentric Castleman's disease: a randomised, double-blind, placebo-controlled trial","volume":"15","author":"van Rhee F","year":"2014","unstructured":"van Rhee F, Wong RS, Munshi N,  Siltuximab for multicentric Castleman's disease: a randomised, double-blind, placebo-controlled trial. Lancet Oncol 2014;15:966\u2013974."},{"key":"B14","series-title":"Blood","first-page":"2115","article-title":"International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease","volume":"132","author":"van Rhee F","year":"2018","unstructured":"van Rhee F, Voorhees P, Dispenzieri A,  International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132:2115\u20132124."},{"key":"B15","series-title":"Am J Hematol","first-page":"1241","article-title":"Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease","volume":"96","author":"Nishimura Y","year":"2021","unstructured":"Nishimura Y, Fajgenbaum DC, Pierson SK,  Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease. Am J Hematol 2021;96:1241\u20131252."},{"key":"B16","series-title":"Blood Adv","first-page":"6652","article-title":"Treatment consistent with idiopathic multicentric Castleman disease guidelines is associated with improved outcomes","volume":"7","author":"Pierson SK","year":"2023","unstructured":"Pierson SK, Lim MS, Srkalovic G,  Treatment consistent with idiopathic multicentric Castleman disease guidelines is associated with improved outcomes. Blood Adv 2023;7:6652\u20136664."},{"key":"B17","series-title":"J Clin Pathol","first-page":"43","article-title":"Diagnostic challenges of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of idiopathic multicentric Castleman disease (iMCD): factors to differentiate from IgG4-related disease","volume":"79","author":"Nishikori A","year":"2025","unstructured":"Nishikori A, Nishimura MF, Fajgenbaum DC,  Diagnostic challenges of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of idiopathic multicentric Castleman disease (iMCD): factors to differentiate from IgG4-related disease. J Clin Pathol 2025;79:43\u201349."},{"key":"B18","series-title":"Haematologica","first-page":"1705","article-title":"Distinct interleukin-6 production in IPL and TAFRO subtypes of idiopathic multicentric Castleman disease","volume":"111","author":"Nishikori A","year":"2026","unstructured":"Nishikori A, Nishimura MF, Nishimura Y,  Distinct interleukin-6 production in IPL and TAFRO subtypes of idiopathic multicentric Castleman disease. Haematologica 2026;111:1705\u20131715."},{"key":"B19","series-title":"Blood Adv","article-title":"Comprehensive analysis of subtype-specific outcomes and management in Castleman disease: a 20-year cohort study","volume":"10","author":"Nishimura Y","year":"2026","unstructured":"Nishimura Y, Habermann TM, Gertz MA,  Comprehensive analysis of subtype-specific outcomes and management in Castleman disease: a 20-year cohort study. Blood Adv 2026;10:1487\u20131496."},{"key":"B20","article-title":"NCCN Clinical Guidelines Practice in Oncology: Castleman Disease. Version 2.2026","author":"Zelenetz AD","unstructured":"Zelenetz AD, Gordon LI, Abramson JS,  NCCN Clinical Guidelines Practice in Oncology: Castleman Disease. Version 2.2026. Accessed May 28, 2026. To view the most recent version, visit https:\/\/www.nccn.org"},{"key":"B21","series-title":"Arthritis Rheumatol","first-page":"12","article-title":"Expert perspective: diagnosis and treatment of Castleman disease","volume":"78","author":"Chen LYC","year":"2026","unstructured":"Chen LYC, Zhang L, Fajgenbaum DC. Expert perspective: diagnosis and treatment of Castleman disease. Arthritis Rheumatol 2026;78:12\u201325."},{"key":"B22","series-title":"Blood","first-page":"1658","article-title":"Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease","volume":"129","author":"Yu L","year":"2017","unstructured":"Yu L, Tu M, Cortes J,  Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease. Blood 2017;129:1658\u20131668."},{"key":"B23","series-title":"Br J Haematol","first-page":"206","article-title":"The full spectrum of Castleman disease: 273 patients studied over 20 years","volume":"180","author":"Oksenhendler E","year":"2018","unstructured":"Oksenhendler E, Boutboul D, Fajgenbaum D,  The full spectrum of Castleman disease: 273 patients studied over 20 years. Br J Haematol 2018;180:206\u2013216."},{"key":"B24","series-title":"Nat Rev Dis Primers","first-page":"84","article-title":"Castleman disease","volume":"7","author":"Carbone A","year":"2021","unstructured":"Carbone A, Borok M, Damania B,  Castleman disease. Nat Rev Dis Primers 2021;7:84."},{"key":"B25","series-title":"Hematol Oncol Clin North Am","first-page":"65","article-title":"Unicentric Castleman Disease","volume":"32","author":"Wong RSM","year":"2018","unstructured":"Wong RSM. Unicentric Castleman Disease. 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Front Immunol 2025;16:1637792."},{"key":"B32","series-title":"Blood","first-page":"2143","article-title":"Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients","volume":"129","author":"Pria AD","year":"2017","unstructured":"Pria AD, Pinato D, Roe J,  Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients. Blood 2017;129:2143\u20132147."},{"key":"B33","series-title":"Int Immunol","first-page":"347","article-title":"IL-6: from its discovery to clinical applications","volume":"22","author":"Kishimoto T","year":"2010","unstructured":"Kishimoto T. IL-6: from its discovery to clinical applications. Int Immunol 2010;22:347\u2013352."},{"key":"B34","series-title":"Annu Rev Immunol","first-page":"1","article-title":"Interleukin-6: from basic science to medicine\u201340 years in immunology","volume":"23","author":"Kishimoto T","year":"2005","unstructured":"Kishimoto T. Interleukin-6: from basic science to medicine\u201340 years in immunology. Annu Rev Immunol 2005;23:1\u201321."},{"key":"B35","series-title":"Blood Adv","first-page":"6534","article-title":"A patient-derived CABIN1 mutation recapitulates features of idiopathic multicentric Castleman disease in a mouse model","volume":"9","author":"Laczko D","year":"2025","unstructured":"Laczko D, Tsao P, Pai RA,  A patient-derived CABIN1 mutation recapitulates features of idiopathic multicentric Castleman disease in a mouse model. Blood Adv 2025;9:6534\u20136537."},{"key":"B36","series-title":"Oncogene","first-page":"3218","article-title":"Genetic basis for iMCD-TAFRO","volume":"39","author":"Yoshimi A","year":"2020","unstructured":"Yoshimi A, Trippett TM, Zhang N,  Genetic basis for iMCD-TAFRO. Oncogene 2020;39:3218\u20133225."},{"key":"B37","series-title":"Am J Hematol","first-page":"220","article-title":"Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease","volume":"91","author":"Iwaki N","year":"2016","unstructured":"Iwaki N, Fajgenbaum DC, Nabel CS,  Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease. Am J Hematol 2016;91:220\u2013226."},{"key":"B38","series-title":"Br J Haematol","first-page":"1830","article-title":"Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: a distinct subtype of iMCD-not otherwise specified with different clinical features and better survival","volume":"204","author":"Gao YH","year":"2024","unstructured":"Gao YH, Liu YT, Zhang MY,  Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: a distinct subtype of iMCD-not otherwise specified with different clinical features and better survival. Br J Haematol 2024;204:1830\u20131837."},{"key":"B39","series-title":"J Clin Pathol","first-page":"1","article-title":"Diagnostic dilemmas in idiopathic multicentric Castleman\u2019s disease idiopathic plasmacytic lymphadenopathy (IPL) type and IgG4-related lymphadenopathy: challenges in recognition and distinction, especially in western pathology","volume":"79","author":"Deshpande V","year":"2025","unstructured":"Deshpande V, Ferry J. Diagnostic dilemmas in idiopathic multicentric Castleman\u2019s disease idiopathic plasmacytic lymphadenopathy (IPL) type and IgG4-related lymphadenopathy: challenges in recognition and distinction, especially in western pathology. J Clin Pathol 2025;79:1\u20133."},{"key":"B40","series-title":"Nat Rev Immunol","first-page":"666","article-title":"Targeting IL-6 trans-signalling: past, present and future prospects","volume":"23","author":"Rose-John S","year":"2023","unstructured":"Rose-John S, Jenkins BJ, Garbers C,  Targeting IL-6 trans-signalling: past, present and future prospects. Nat Rev Immunol 2023;23:666\u2013681."},{"key":"B41","series-title":"Haematologica","first-page":"207","article-title":"The lymph node transcriptome of unicentric and idiopathic multicentric Castleman disease","volume":"108","author":"Horna P","year":"2023","unstructured":"Horna P, King RL, Jevremovic D,  The lymph node transcriptome of unicentric and idiopathic multicentric Castleman disease. Haematologica 2023;108:207\u2013218."},{"key":"B42","series-title":"Lancet Haematol","first-page":"e163","article-title":"Idiopathic multicentric Castleman's disease: a systematic literature review","volume":"3","author":"Liu AY","year":"2016","unstructured":"Liu AY, Nabel CS, Finkelman BS,  Idiopathic multicentric Castleman's disease: a systematic literature review. Lancet Haematol 2016;3:e163\u2013175."},{"key":"B43","series-title":"Adv Exp Med Biol","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1007\/978-981-10-5765-6_7","article-title":"Kaposi's sarcoma-associated herpesvirus: epidemiology and molecular biology","volume":"1018","author":"Li S","year":"2017","unstructured":"Li S, Bai L, Dong J,  Kaposi's sarcoma-associated herpesvirus: epidemiology and molecular biology. Adv Exp Med Biol 2017;1018:91\u2013127."},{"key":"B44","series-title":"Blood","first-page":"1186","article-title":"Kaposi sarcoma-associated herpesvirus\/human herpesvirus 8-associated lymphoproliferative disorders","volume":"133","author":"Oksenhendler E","year":"2019","unstructured":"Oksenhendler E, Boutboul D, Galicier L. Kaposi sarcoma-associated herpesvirus\/human herpesvirus 8-associated lymphoproliferative disorders. Blood 2019;133:1186\u20131190."},{"key":"B45","series-title":"J Virol","first-page":"e00909","article-title":"Genetic analyses of contributions of viral interleukin-6 interactions and signaling to human herpesvirus 8 productive replication","volume":"94","author":"Li Q","year":"2020","unstructured":"Li Q, Xiang Q, Chen D, Nicholas J. Genetic analyses of contributions of viral interleukin-6 interactions and signaling to human herpesvirus 8 productive replication. J Virol 2020;94:e00909\u201300920."},{"key":"B46","series-title":"Eur J Haematol","first-page":"309","article-title":"Efficacy and safety of regimens used for the treatment of multicentric Castleman disease: a systematic review","volume":"109","author":"Rehman MEU","year":"2022","unstructured":"Rehman MEU, Chattaraj A, Neupane K,  Efficacy and safety of regimens used for the treatment of multicentric Castleman disease: a systematic review. Eur J Haematol 2022;109:309\u2013320."},{"key":"B47","series-title":"Nat Rev Clin Oncol","first-page":"533","article-title":"Castleman's disease\u2013a two compartment model of HHV8 infection","volume":"7","author":"Schulte KM","year":"2010","unstructured":"Schulte KM, Talat N. Castleman's disease\u2013a two compartment model of HHV8 infection. Nat Rev Clin Oncol 2010;7:533\u2013543."},{"key":"B48","series-title":"J Clin Invest","first-page":"3165","article-title":"Kaposi sarcoma-associated herpesvirus: immunobiology, oncogenesis, and therapy","volume":"126","author":"Dittmer DP","year":"2016","unstructured":"Dittmer DP, Damania B. Kaposi sarcoma-associated herpesvirus: immunobiology, oncogenesis, and therapy. J Clin Invest 2016;126:3165\u20133175."},{"key":"B49","series-title":"Am J Hematol","first-page":"E15","article-title":"Siltuximab administration results in spurious IL-6 elevation in peripheral blood","volume":"99","author":"Mango NA","year":"2024","unstructured":"Mango NA, Pierson SK, Sarmiento Bustamante M,  Siltuximab administration results in spurious IL-6 elevation in peripheral blood. 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Am J Hematol 2026;101:165\u2013168."},{"key":"B51","series-title":"Mod Rheumatol","first-page":"456","article-title":"Effectiveness and safety of tocilizumab, an anti-interleukin-6 receptor antibody, in 342 Japanese patients with multicentric Castleman disease: a 3-year, prospective, observational study","volume":"36","author":"Nishimoto N","year":"2026","unstructured":"Nishimoto N, Murakami M, Johkoh T,  Effectiveness and safety of tocilizumab, an anti-interleukin-6 receptor antibody, in 342 Japanese patients with multicentric Castleman disease: a 3-year, prospective, observational study. Mod Rheumatol 2026;36:456\u2013468."},{"key":"B52","series-title":"J Clin Exp Hematop","first-page":"69","article-title":"Clinical features and treatment of multicentric Castleman's disease: a retrospective study of 21 Japanese patients at a single institute","volume":"53","author":"Kawabata H","year":"2013","unstructured":"Kawabata H, Kadowaki N, Nishikori M,  Clinical features and treatment of multicentric Castleman's disease: a retrospective study of 21 Japanese patients at a single institute. J Clin Exp Hematop 2013;53:69\u201377."},{"key":"B53","series-title":"Ann Rheum Dis","first-page":"1580","article-title":"Long-term safety and efficacy of tocilizumab, an anti-IL-6 receptor monoclonal antibody, in monotherapy, in patients with rheumatoid arthritis (the STREAM study): evidence of safety and efficacy in a 5-year extension study","volume":"68","author":"Nishimoto N","year":"2009","unstructured":"Nishimoto N, Miyasaka N, Yamamoto K,  Long-term safety and efficacy of tocilizumab, an anti-IL-6 receptor monoclonal antibody, in monotherapy, in patients with rheumatoid arthritis (the STREAM study): evidence of safety and efficacy in a 5-year extension study. Ann Rheum Dis 2009;68:1580\u20131584."},{"key":"B54","series-title":"Ann Hematol","first-page":"6229","article-title":"Siltuximab monotherapy improves progression free survival compared to rituximab-based therapies in patients with idiopathic multicentric Castleman disease; indirect comparison of studies using single-arm metanalysis method and the generalized linear mixed model","volume":"104","author":"Karatisidis L","year":"2025","unstructured":"Karatisidis L, Mprotsis T, Intzes S,  Siltuximab monotherapy improves progression free survival compared to rituximab-based therapies in patients with idiopathic multicentric Castleman disease; indirect comparison of studies using single-arm metanalysis method and the generalized linear mixed model. Ann Hematol 2025;104:6229\u20136235."},{"key":"B55","series-title":"Ann Hematol","first-page":"1641","article-title":"Effectiveness of rituximab-containing treatment regimens in idiopathic multicentric Castleman disease","volume":"97","author":"Dong Y","year":"2018","unstructured":"Dong Y, Zhang L, Nong L,  Effectiveness of rituximab-containing treatment regimens in idiopathic multicentric Castleman disease. Ann Hematol 2018;97:1641\u20131647."},{"key":"B56","series-title":"Blood Rev","first-page":"101161","article-title":"Idiopathic multicentric Castleman disease: an update in diagnosis and treatment advances","volume":"64","author":"Lang E","year":"2024","unstructured":"Lang E, van Rhee F. Idiopathic multicentric Castleman disease: an update in diagnosis and treatment advances. Blood Rev 2024;64:101161."},{"key":"B57","series-title":"N Engl J Med","first-page":"2255","article-title":"Cytokine storm","volume":"383","author":"Fajgenbaum DC","year":"2020","unstructured":"Fajgenbaum DC, June CH. Cytokine storm. N Engl J Med 2020;383:2255\u20132273."},{"key":"B58","series-title":"Blood","first-page":"1720","article-title":"Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease","volume":"133","author":"Zhang L","year":"2019","unstructured":"Zhang L, Zhao AL, Duan MH,  Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease. Blood 2019;133:1720\u20131728."},{"key":"B59","series-title":"Ann Hematol","first-page":"1713","article-title":"Real-world data of siltuximab for Chinese patients with iMCD: combination with BCD regimen as a potential approach for severe cases","volume":"104","author":"Li SY","year":"2025","unstructured":"Li SY, Gao YH, Dang Y,  Real-world data of siltuximab for Chinese patients with iMCD: combination with BCD regimen as a potential approach for severe cases. Ann Hematol 2025;104:1713\u20131720."},{"key":"B60","series-title":"Ann Hematol","first-page":"4223","article-title":"Sirolimus is effective for refractory\/relapsed idiopathic multicentric Castleman disease: a single-center, retrospective study","volume":"103","author":"Liu YT","year":"2024","unstructured":"Liu YT, Gao YH, Zhao H,  Sirolimus is effective for refractory\/relapsed idiopathic multicentric Castleman disease: a single-center, retrospective study. Ann Hematol 2024;103:4223\u20134230."},{"key":"B61","series-title":"N Engl J Med","first-page":"616","article-title":"Identifying and targeting TNF signaling in idiopathic multicentric Castleman's disease","volume":"392","author":"Mumau MD","year":"2025","unstructured":"Mumau MD, Gonzalez MV, Ma C,  Identifying and targeting TNF signaling in idiopathic multicentric Castleman's disease. N Engl J Med 2025;392:616\u2013618."},{"key":"B62","series-title":"Nat Commun","first-page":"7236","article-title":"CXCL13 is a predictive biomarker in idiopathic multicentric Castleman disease","volume":"13","author":"Pierson SK","year":"2022","unstructured":"Pierson SK, Katz L, Williams R,  CXCL13 is a predictive biomarker in idiopathic multicentric Castleman disease. 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