A Novel Predictive Model for Idiopathic Multicentric Castleman Disease: The International Castleman Disease Consortium Study

The oncologist

article

Li Yu¹  Ruifang Sun³  Shanxiang Zhang⁴  Hanjin Yang⁵  Zhaoming Wang⁵  Wenbin Qian⁶  Noriko Iwaki⁷  Yasuharu Sato⁸  Lu Zhang⁹  Jian Li⁹  Eric Oksenhendler^1,10  Menghan Shi¹  Zijun Y. Xu-Monette²  Ken H. Young²  Qingqing Cai^1,12  Paolo Strati^1,13  Fredrick Hagemeister^1,13  Qiongli Zhai^1,14  Ling Li^1,15  Xiaosheng Fang^1,16  Jianyong Li^1,17 

[1] Department of Hematology and Oncology, The Second Affiliated Hospital of NanChang University;Division of Hematopathology and Department of Pathology, Duke University Medical Center;Department of Pathology, Shanxi Cancer Hospital;Department of Pathology, Indiana University School of Medicine;Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine;Department of Hematology and Oncology, The Second Affiliated Hospital, Zhejiang University School of Medicine;Department of Hematology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University;Division of Pathophysiology, Okayama University Graduate School of Health Sciences;Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College;Département d'Immunologie Clinique, Hôpital Saint-Louis;Duke Cancer Institute;State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Sun Yat-sen University Cancer Center;Departments of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center;Department of Pathology, Tianjin Medical University Cancer Institute and Hospital;Department of Medical Oncology, Zhengzhou University Cancer Center;Department of Hematology, Shandong Provincial Hospital Affiliated to Shandong First Medical University;Department of Hematology and Oncology, Jiangsu Province Hospital, Nanjing University

关键词: Castleman disease;    Idiopathic multicentric Castleman disease;    Prognosis;    Treatment;    Risk stratification;    International prognostic index;   

DOI  :  10.1634/theoncologist.2019-0986

学科分类：地质学

来源: AlphaMed Press Incorporated

【 摘 要 】

Background 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 “watchful waiting” to high-dose chemotherapy. This diverse clinical presentation calls for a classification stratification system that takes into account the severity of the disease. Subjects, Materials, and Methods We analyzed the clinical, laboratory, and pathologic abnormalities and treatment outcomes of 176 patients with iMCD (median follow-up duration 12 years) 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. Results Using these data, we proposed and validated the iMCD international prognostic index (iMCD-IPI), which includes parameters related to patient characteristics (age > 40 years), histopathologic features (plasma cell variant), and inflammatory consequences of iMCD (hepatomegaly and/or splenomegaly, hemoglobin 40 years, plasmacytic variant subtype, hepatomegaly and/or splenomegaly, hemoglobin <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.

【 授权许可】

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