Clinical Analysis of Unicentric Castleman's Disease with Paraneoplastic Pemphigus and Bronchiolitis Obliterans.

Objective To investigate the clinical features of unicentric Castleman's disease(UCD)with paraneoplastic pemphigus(PNP)and bronchiolitis obliterans(BO).Method Data of UCD patients with PNP and BO from Peking Union Medical College Hospital were retrospectively analyzed,along with literatures review. Results Totally 23 cases(11 males and 12 females)were enrolled.The median age was 31 years(13-56 years).The most common pathological type was hyaline-vascular variant(91.4%),and most tumors located in abdominopelvic cavity(69.6%).Considerable cases presented bulky masses(26.3%).Most cases were first diagnosed on presentation with the symtoms of PNP(90.0%).BO was characterized by progressive dyspnea after excision of CD lesions.The average follow-up duration was 27.5 months(1-135 months).The median overall survival time was 36.0 months(95% CI=13.9-58.1).Respiratory failure was the dominant cause of death(91.7%).Conclusions PNP should be considered among those patients with specific oral or cutaneous lesions.Earlier diagnosis and treatment of latent UCD are important for reducing complications and deaths.