A Real-world Study on a Large Retrospective Cohort of Castleman Disease from a Single Center

Abstract

Castleman disease(CD) is a rare lymphoid tissue proliferative disease with an increasing focus on etiology and treatment in recent years. The consensus on CD diagnosis and treatment has been published by several organizations, playing an important role in promoting cooperation on CD managements and investigations among different teams. Based on the last 15-years retrospective real-world data from Peking University First Hospital (PKUFH), we re-classified and re-evaluated the clinical and pathological information of patients with pathologically suspected diagnosis of CD. A total of 203 patients were included in our study, in which the diagnosis of CD was confirmed in 189 cases, including 118 patients with unicentric CD (UCD, n = 118, 62.4%) and 71 patients with multicentric CD (MCD, n = 71, 37.6%). 44.1% (n = 52) of UCDs in our cohort were complicated with Paraneoplastic Pemphigus (PNP). The treatment of UCD is mostly surgical resection, with a 5-year overall survival(OS) 88.1%. Patients with PNP had a poorer prognosis than those without PNP [82.9% (95% CI 123–178) vs 92.8% (95% CI 168–196), log-rank P = 0.041). The rate of concurrent systemic symptoms was 74.6% (n = 53), and renal involvement occurred in 49.3% (n = 35) MCD patients. The MCD treatments were mainly chemotherapy regimens, with a 5-year OS of 77.6% (95% CI, 143–213). In conclusion, UCDs have a better overall prognosis than MCDs. But the prognosis of those complicated with PNP was poor. Differential diagnosis of MCD is difficult. MCD treatment in China is heterogeneous. The inaccessibility of anti-IL-6-targeted drugs in China may contribute to the poor prognosis of MCD.