title: Clinical and laboratory characterization of 114 cases of Castleman disease patients from a single centre: paraneoplastic pemphigus is an unfavourable prognostic factor.
abstract: This study retrospectively collected the clinical and laboratory data of 114 patients with Castleman disease (CD) from a single medical centre. Clinical classification identified 62 patients (54·4%) with unicentric Castleman disease and 52 (45·6%) with multi-centric Castleman disease. Pathological classification revealed 68 cases (59·6%) of hyaline vascular variant, 16 (14·1%) mixed cellular variant (Mix) and 30 (26·3%) plasmacytic variant. Clinical complications occurred in 69 CD patients, including 37 cases of paraneoplastic pemphigus (PNP) and 25 cases with renal complications. Haematological involvement, pleural effusion and/or ascites and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) were also found. Univariate analysis showed that presence of clinical complications and PNP were both risk factors relating to CD patient survival. Prognostic factors showing P < 0·15 in univariate analysis and those with clinical significance were subjected to multivariate analysis using a Cox regression model. PNP presence and age over 40 years both significantly adversely affected survival. Thus, only presence of PNP was identified as an independent unfavourable survival risk factor in both univariate and multivariate analyses. Overall, the present data provide a panoramic description of CD cases and emphasize that the presence of PNP is an adverse prognostic factor.
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Yujun
authors.initials: Y
authors.lastname: Dong
authors.affiliation: Department of Dermatology, Peking University First Hospital, Beijing, China.
authors.forename: Mingyue
authors.initials: M
authors.lastname: Wang
authors.affiliation: Department of Pathology, Peking University First Hospital, Beijing, China.
authors.forename: Lin
authors.initials: L
authors.lastname: Nong
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Lihong
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Xinan
authors.initials: X
authors.lastname: Cen
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Liu
authors.affiliation: Department of Biostatistics, Peking University First Hospital, Beijing, China.
authors.forename: Sainan
authors.initials: S
authors.lastname: Zhu
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Yuhua
authors.initials: Y
authors.lastname: Sun
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Zeyin
authors.initials: Z
authors.lastname: Liang
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Jinping
authors.initials: J
authors.lastname: Ou
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Zhixiang
authors.initials: Z
authors.lastname: Qiu
authors.affiliation: Department of Haematology, Peking University First Hospital, Beijing, China.
authors.forename: Hanyun
authors.initials: H
authors.lastname: Ren
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D005871:Castleman Disease / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D018858:Germinal Center / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000209:etiology; D010392:Pemphigus / Q000175:diagnosis / Q000209:etiology; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
keywords: Castleman disease; clinical complications; paraneoplastic pemphigus; prognostic factors; survival analysis