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A national, multicenter, retrospective study of Castleman disease in China implementing CDCN criteria

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机构: [1]Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. [2]State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. [3]Department of Haematology, Peking University First Hospital, Beijing, China. [4]Department of Haematology, Second Xiang-ya Hospital, Central South University, Changsha, China. [5]Department of Haematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China. [6]Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. [7]Department of Haematology, Sheng Jing Hospital of China Medical University, Shenyang, China. [8]Department of Haematology, The First Affiliated Hospital of Anhui Medical University, Hefei, China. [9]Department of Haematology, Shanxi Province Cancer Hospital, Taiyuan, China. [10]Department of Haematology, Guangdong Provincial People's Hospital/Guangdong Academy of Medical Sciences, Guangzhou, China. [11]Bone Marrow Transplantation Center, The First Affiliated Hospital of Zhejiang Medical College, Zhejiang University, Hangzhou, China. [12]Department of Haematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. [13]Department of Haematology, First Hospital of China Medical University, Shenyang, China. [14]Department of Haematology, Jiangsu Province Hospital and Nanjing Medical University First Affiliated Hospital, Nanjing, China. [15]Department of Haematology, West China Hospital Sichuan University, Chengdu, China. [16]Department of Haematology, China-Japan Friendship Hospital, Beijing, China. [17]Department of Haematology and Oncology, China-Japan Union Hospital of Jilin University, Changchun, China. [18]Department of Haematology, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, China. [19]Department of Haematology, Xijing Hospital, Fourth Military Medical University, Xi'an, China. [20]Department of Haematology, Xinqiao Hospital, Army Medical University, Chongqing, China. [21]Department of Haematology, Fujian Medical University Union Hospital, Fuzhou, China. [22]Department of Haematology, Nanfang Hospital, Southern Medical University, Guangzhou, China. [23]Department of Haematology/Oncology, Chongqing University Cancer Hospital, Chongqing Cancer Hospital, Chongqing, China. [24]Department of Haematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China. [25]Department of Lymphoma & Haematology, Hunan Cancer Hospital/The Affiliated Tumor Hospital of Xiangya Medical School, Central South University, Changsha, China. [26]Institute of Haematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. [27]Department of Haematology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China. [28]Department of Lymphoma and Myeloma, State Key Laboratory of Experimental Haematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Haematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China. [29]Department of Lymphoma, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China. [30]Department of Haematology, The First Affiliated Hospital of USTC, Hefei, China. [31]Department of Haematology, The Second Hospital of Dalian Medical University, Dalian, China. [32]Department of Haematology, The First Hospital of Jilin University, Changchun, China. [33]Department of Haematology, The Fifth Medical Center of Chinese PLA General Hospital, Beijing, China. [34]Department of Haematology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China. [35]Department of Haematology, Second Hospital of Hebei Medical University, Shijiazhuang, China. [36]Department of Haematology, Tianjin Medical University General Hospital, Tianjin, China. [37]Department of Haematology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China. [38]Department of Haematology, Huashan Hospital, Fudan University, Shanghai, China. [39]Department of Haematology, The First Affiliated Hospital of Guangxi Medical University, Nanning, China. [40]Department of Haematology, The First Affiliated Hospital of Harbin Medical University, Harbin, China. [41]Department of Haematology, Shanxi Bethune Hospital, Taiyuan, China. [42]Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
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关键词: Castleman disease Multicenter study Treatment options Survival analysis

摘要:
Castleman disease (CD) is a group of rare and heterogenous lymphoproliferative disorders including unicentric CD (UCD), human herpesvirus-8(HHV-8)-associated multicentric CD (HHV8-MCD), and HHV-8-negative/idiopathic multicentric CD (iMCD). Knowledge of CD mainly comes from case series or retrospective studies, but the inclusion criteria of these studies vary because the Castleman Disease Collaborative Network (CDCN) diagnostic criteria for iMCD and UCD were not available until 2017 and 2020, respectively. Further, these criteria and guidelines have not been systematically evaluated.In this national, multicenter, retrospective study implementing CDCN criteria, we enrolled 1634 CD patients (UCD, n = 903; MCD, n = 731) from 2000 to 2021 at 40 Chinese institutions to depict clinical features, treatment options, and prognostic factors of CD.Among UCD, there were 162 (17.9%) patients with an MCD-like inflammatory state. Among MCD, there were 12 HHV8-MCD patients and 719 HHV-8-negative MCD patients, which included 139 asymptomatic MCD (aMCD) and 580 iMCD meeting clinical criteria. Of 580 iMCD patients, 41 (7.1%) met iMCD-TAFRO criteria, the others were iMCD-NOS. iMCD-NOS were further divided into iMCD-IPL (n = 97) and iMCD-NOS without IPL (n = 442). Among iMCD patients with first-line treatment data, a trend from pulse combination chemotherapy toward continuous treatment was observed. Survival analysis revealed significant differences between subtypes and severe iMCD (HR = 3.747; 95% CI: 2.112-6.649, p < 0.001) had worse outcome.This study depicts a broad picture of CD, treatment options and survival information in China and validates the association between the CDCN's definition of severe iMCD and worse outcomes, requiring more intensive treatment.Beijing Municipal Commission of Science and Technology, CAMS Innovation Fund and National High Level Hospital Clinical Research Funding.© 2023 Published by Elsevier Ltd.

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出版当年[2023]版:
大类 | 1 区 医学
小类 | 1 区 卫生保健与服务 1 区 公共卫生、环境卫生与职业卫生
最新[2023]版:
大类 | 1 区 医学
小类 | 1 区 卫生保健与服务 1 区 公共卫生、环境卫生与职业卫生
第一作者:
第一作者机构: [1]Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. [2]State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
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通讯作者:
通讯机构: [1]Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. [2]State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. [*1]Department of Haematology and State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, China.
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