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.