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

Abstract

Objective: Castleman disease (CD) is a rare lymphoproliferative disorder with limited clinical research data. This study aimed to investigate the clinical manifestations, pathologic features, and prognostic factors of CD.

Methods: The clinicopathological data of 54 patients with CD hospitalized in the First Affiliated Hospital of Nanjing Medical University were retrospectively analyzed. Univariate analysis and multivariate analysis were performed by Cox regression model to determine independent prognostic factors for patients' survival.

Results: According to clinical classification, 30 cases (55.6%) had unicentric CD (UCD) and 24 cases (44.4%) had multicentric CD (MCD). Moreover, pathologic classification revealed 32 cases (59.3%) with hyaline vascular variant, 3 (5.6%) with mixed cellular variant, and 19 (35.2%) with plasmacytic variant. Patients with MCD commonly presented with clinical signs and symptoms, including fever, splenomegaly, and pleural effusion and/or ascites. Clinical complications, such as liver injury, anemia, and polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome were more common in patients with MCD. Univariate analysis showed that the presence of paraneoplastic pemphigus (PNP) and the elevation of C-reactive protein were unfavorable prognosticators of survival in patients with CD. By multivariate analysis PNP was an independent prognostic factor in patients with CD.

Conclusions: This study provided a panoramic elaboration of CD cases and showed that the presence of PNP was an independent unfavorable factor.

Keywords: Castleman disease; paraneoplastic pemphigus; prognostic factors; survival.

Figure 1

Histopathological features of different variants of Castleman disease. A. Hyaline-vascular variant. The germinal center of a follicle is penetrated by a hyalinized blood vessel, resembling a lollipop and surrounded by a mantle zone composed of lymphocytes in an “onion skin” pattern. B. Plasmacytic variant. The interfollicular region contains sheets of mature plasma cells. C. Mixed cellular variant. The histopathologic features were intermediate between Hyaline-vascular variant and Plasmacytic variant.

Figure 2

Kaplan-Meier survival analysis of 54 patients with Castleman disease. Log-rank regression was used to test the significance between the two groups. A. The survival rate of CD patients with elevated CRP was significantly lower than that of patients with normal CRP level. B. Prognosis of Castleman disease patients with PNP was worse than that of patients without PNP. CRP, C-reaction protein; PNP, paraneoplastic pemphigus.