{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T12:30:18Z","timestamp":1784205018042,"version":"3.55.0"},"reference-count":24,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2020,9,18]],"date-time":"2020-09-18T00:00:00Z","timestamp":1600387200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Patients with multicentric Castleman disease (MCD) who are negative for human immunodeficiency virus and human herpesvirus 8 are considered to have idiopathic MCD (iMCD). The clinical presentation of iMCD varies from mild constitutional symptoms to life-threatening symptoms or death. The treatment strategy varies from \u201cwatchful waiting\u201d to high-dose chemotherapy. This diverse clinical presentation calls for a classification stratification system that takes into account the severity of the disease.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Subjects, Materials, and Methods<\/jats:title>\n                  <jats:p>We analyzed the clinical, laboratory, and pathologic abnormalities and treatment outcomes of 176 patients with iMCD (median follow-up duration 12\u2009years) from the U.S. and China to better understand the characteristics and prognostic factors of this disease. This discovery set of iMCD results was confirmed from the validation set composed of additional 197 patients with iMCD organized from The International Castleman Disease Consortium.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Using these data, we proposed and validated the iMCD international prognostic index (iMCD-IPI), which includes parameters related to patient characteristics (age &amp;gt;\u200940\u2009years), histopathologic features (plasma cell variant), and inflammatory consequences of iMCD (hepatomegaly and\/or splenomegaly, hemoglobin &amp;lt;80 g\/L, and pleural effusion). These five factors stratified patients according to their performance status and extent of organ dysfunction into three broad categories: low risk, intermediate risk, and high risk. The iMCD-IPI score accurately predicted outcomes in the discovery study cohort, and the results were confirmed on the validation study cohort.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>This study represents the largest series of studies on patients with iMCD in the field and proposed a novel risk-stratification model for iMCD-IPI that could be used to guide risk-stratified treatment strategies in patients with iMCD.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Implications for Practice<\/jats:title>\n                  <jats:p>Patients with idiopathic multicentric Castleman disease (iMCD) can benefit from care based on clinical symptoms and disease severity. This study in 176 patients with iMCD constructed an iMCD-IPI score based on five clinical factors, including age &amp;gt;40\u2009years, plasmacytic variant subtype, hepatomegaly and\/or splenomegaly, hemoglobin &amp;lt;80 g\/L, and pleural effusion, and stratified patients into three risk categories: low risk, intermediate risk, and high risk. The predictive value was validated in an independent set of 197 patients with iMCD from The International Castleman Disease Consortium. The proposed novel model is valuable for predicting clinical outcome and selecting optimal therapies using clinical parameters.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1634\/theoncologist.2019-0986","type":"journal-article","created":{"date-parts":[[2020,9,21]],"date-time":"2020-09-21T16:08:01Z","timestamp":1600704481000},"page":"963-973","source":"Crossref","is-referenced-by-count":15,"title":["A Novel Predictive Model for Idiopathic Multicentric Castleman Disease: The International Castleman Disease Consortium Study"],"prefix":"10.1093","volume":"25","author":[{"given":"Li","family":"Yu","sequence":"first","affiliation":[{"name":"Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University, NanChang, People's Republic of China"},{"name":"Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Menghan","family":"Shi","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University, NanChang, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qingqing","family":"Cai","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Strati","sequence":"additional","affiliation":[{"name":"Departments of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fredrick","family":"Hagemeister","sequence":"additional","affiliation":[{"name":"Departments of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiongli","family":"Zhai","sequence":"additional","affiliation":[{"name":"Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, Tianjin, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ling","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Zhengzhou University Cancer Center, Zhengzhou, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaosheng","family":"Fang","sequence":"additional","affiliation":[{"name":"Department of Hematology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Ji'nan, Shandong, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianyong","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology, Jiangsu Province Hospital, Nanjing University, Nanjing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruifang","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Pathology, Shanxi Cancer Hospital, Taiyuan, Shanxi, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanxiang","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Indiana University School of Medicine, Indianapolis, Indiana, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanjin","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhaoming","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenbin","family":"Qian","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriko","family":"Iwaki","sequence":"additional","affiliation":[{"name":"Department of Hematology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuharu","family":"Sato","sequence":"additional","affiliation":[{"name":"Division of Pathophysiology, Okayama University Graduate School of Health Sciences, Okayama, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Oksenhendler","sequence":"additional","affiliation":[{"name":"D\u00e9partement d'Immunologie Clinique, H\u00f4pital Saint-Louis, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zijun Y.","family":"Xu-Monette","sequence":"additional","affiliation":[{"name":"Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken H.","family":"Young","sequence":"additional","affiliation":[{"name":"Division of Hematopathology and Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA"},{"name":"Duke Cancer Institute, Durham, North Carolina, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,9,18]]},"reference":[{"key":"2021122511250769400_onco13493-bib-0001","volume-title":"Weekly Clinicopathological Exercises. 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