Clinical characteristics and prognosis of castleman disease patients in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor

Abstract

Background Castleman disease (CD) is a rare lymphoproliferative disorder that has had limited clinical research. This study aims to detect the clinical manifestations, pathological features, and prognostic factors of this disease.Methods This study retrospectively analyzed the information of 54 patients with CD hospitalized in a single centre. A Cox regression model was employed to perform univariate analysis and multivariate analysis in order to identify independent prognostic factors for survival.Results Based on clinical classification, 26 patients (55.6%) had unicentric CD (UCD) and 28 patients (44.4%) had multicentric CD (MCD). Moreover, pathological classification identified 32 cases (59.3%) with hyaline vascular variant (HV), 3 (5.6%) with mixed cellular variant (Mix), and 19 (35.2%) with plasmacytic variant (PC). The MCD patients commonly exhibited clinical signs and symptoms, including fever, splenomegaly, and pleural effusion and/or ascites. Several clinical complications, such as liver injury, anemia, and polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes syndrome (POEMS) were more common in MCD patients. Univariate analysis showed that presence of paraneoplastic pemphigus (PNP) and elevated C-reactive protein (CRP) were unfavorable factors relating to CD patient survival. Multivariate analysis identified the presence of PNP as an independent prognostic factor in patients with CD.Conclusions This study provided a panoramic elaboration of CD cases and showed the presence of PNP was an independent unfavorable factor.

Paraneoplastic pemphigus: Diagnostic mimics, confounders and management challenges in a series of five long‐term survivors from a single centre

“…As demonstrated in this small series, early clinical suspicion, extensive imaging to investigate for often clinically silent malignancy, intensive multidisciplinary care, awareness for and management of BO, if present, resulted in impressive long‐term survival when compared with recent data of less than 50% survival at 6 years 12 …”