The clinical, pathological treatment, and prognostic data of 55 patients with Castleman disease (CD) were collected retrospectively from a single medical center. Thirty-four cases were classified as uni-centric; the remaining 21 cases were diagnosed as multi-centric CD. Regarding pathological classification, 38 cases were hyaline vascular type, account for 69.1% of all patients. Nine cases were diagnosed as plasmacytic type and eight cases as mixed cellularity type. Several prominent clinical complications were noted in this group of patients with CD: the skin, internal organs, and hematopoietic system were involved individually or concurrently. Clinical complications were distinct between different clinical and pathological subtypes of CD. The unique clinical and laboratory features of patients with paraneoplastic pemphigus suggests this diagnosis should be characterized as an independent disease entity. The presence of clinical complications is an independent prognostic factor in all patients with CD. More effective initial therapy should be considered in patients with CD with complications to improve the overall survival.
Background: Castleman tumor, a rare lymphoproliferative disorder, is one of the associated tumors in paraneoplastic pemphigus. We analyzed the characteristics of a group of patients with Castleman tumor to clearly understand and to improve the prognosis of the disease.Observations: Ten cases of paraneoplastic pemphigus associated with Castleman tumor treated in the Department of Dermatology, Peking University First Hospital, Beijing, China, from May 1, 1999, to March 31, 2004, were analyzed for clinical aspects, characteristics and histologic features of the tumors, and computed tomographic findings. Literature was reviewed and data were compared with our cases. Castleman tumor was a frequently reported neoplasm in association with paraneoplastic pemphigus in China. The disease was found to be caused by an autoimmune reaction originating from the B lymphocytes in the Castleman tumor.Conclusions: Castleman tumor in association with paraneoplastic pemphigus is a commonly reported subtype of paraneoplastic pemphigus in China. Early detection and removal of the Castleman tumor are crucial for the treatment of this tumor-associated autoimmune disease.
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