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Clinical features analysis of Castleman disease patients
Received:August 03, 2021  Revised:November 19, 2021  Click here to download the full text
Citation of this paper:GU Shi-yang,WEI Zheng,JI Li-li,WANG Zhi-mei,ZHUANG Jing-li,CHENG Zhi-xiang,WANG Wei-guang,YUAN Ling,CHENG Lu-ya,KE Yang,LIU Peng.Clinical features analysis of Castleman disease patients[J].Chinese Journal of Clinical Medicine,2022,29(4):610-614
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Author NameAffiliationE-mail
GU Shi-yang Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
WEI Zheng Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
JI Li-li Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
WANG Zhi-mei Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
ZHUANG Jing-li Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
CHENG Zhi-xiang Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
WANG Wei-guang Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
YUAN Ling Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
CHENG Lu-ya Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
KE Yang Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China  
LIU Peng Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai 200032, China liu.peng@zs-hospital.sh.cn 
Abstract:Objective To analyze clinical pathological and therapeutic features of Castleman disease (CD). Methods Totally, 95 patients were enrolled and their clinical features, lablatory results, pathological features, treatment and survival data were collected. Results Among the CD patients, hyaline vascular pathological subtype accounted for 58.9% (56/95); Uni-center CD (UCD) accounted for 72.6% (69/95) while muitl-center CD (MCD) accounted for 27.4% (26/95). Twenty-one patients had idiopathic MCD-not otherwise specified (iMCD-NOS) subtype. There were significant differences in the pathological subtype, maximum diameter of lesions, the ratios of B symptoms, splenomegaly, and serous effusion, and several laboratory factors between UCD and MCD subgroup (P<0.05). Laboratory examination showed lymphocytopenia happened in 39(41.1%) patients.UCD and asymptomatic MCD (aMCD) patients were free from systemic medication,while iMCD patients were undertaken systemic medication including glucocorticoid, immunomodulatory drugs, rituximab and/or chemotherapy. For all the patients, estimated 3-year progression-free survival (PFS) and overall survival (OS) rates were 78.8% and 92.2%; While for MCD subgroup, estimated 3-year OS and 5-year OS rates were 78.2% and 70.2%. Conclusions The proportions of splenomegaly and serous effusion are low in CD patients, while the lymphocytes decreases, and estimated 5-year OS rate in MCD patients is better than previously reports.
keywords:Castleman disease  idiopathic MCD-not otherwise specified subtype  lymphocytopenia  therapy  survival rate
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