[Clinical features and prognosis in 49 patients with Castleman's disease].
OBJECTIVE
To analyze the clinical features and prognosis of patients with Castleman's disease (CD).


METHODS
Clinical and pathological data of 49 patients with CD diagnosed in Peking Union Medical College Hospital from January 1990 to December 2007 were retrospectively analyzed.


RESULTS
In patients with uni-centric CD (UCD), hyaline vascular type had the highest percentage (88.2%, 15/17), which was significantly higher than that of either plasma cell type (5.9%, 1/17) or mixed cell type (5.9%, 1/17) (P < 0.05). In patients with multicentric CD (MCD), there were no significant differences among the percentages of different histopathologic types. In contrast to patients with UCD, patients with MCD were relatively older and had more typical clinical features, more frequent complications, and more frequent abnormal laboratory results. Twenty patients with UCD achieved complete remission (CR) after surgery, and their complications also disappeared one month later. Twenty-three out of 29 patients with MCD were treated with chemotherapy; only 6 patients achieved CR and 9 achieved partial remission (PR), and the overall response rate was 65.2%. Two patients who initially did not responded to chemotherapy achieved CR after the addition of rituximab.


CONCLUSIONS
The clinical features of CD are multifarious and nonspecific, and diagnosis is exclusively depended on histopathology. UCD has a good prognosis after surgery, while MCD often poorly responds to chemotherapy and has a relatively poor prognosis. New drugs and clinical trials are needed to improve the outcome of MCD.
Yu
Guo-Pan
GP
Department of Internal Medicine, PUMC Hospital, CAMS and PUMC, Beijing 100730, China.
Wang
Shu-Jie
SJ
Shi
Jie
J
Zhao
Yong-Qiang
YQ
D000293:Adolescent; D000328:Adult; D000368:Aged; D005871:Castleman Disease / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult