Treatment and Outcome of Castleman Disease: A Retrospective Report of 31 Patients

BACKGROUND: Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with a spectrum of characteristic pathological abnormalities of lymph node. Furthermore, its clinical diagnosis is very challenging until pathological results are available. This study aimed to investigate the clinical presentations, treatment and prognosis of CD, thereby improving the understanding and diagnosis of CD. METHODS: This study retrospectively analyzed the clinical data of 31 patients with CD admitted to the First Hospital Affiliated Hospital of Chongqing Medical University January 2013 to December 2020. The chi-square test and the Mann–Whitney rank sum test were employed to calculate between-group differences for categorical and quantitative data, respectively. RESULTS: Clinically, patients with unicentric CD (UCD) usually present with lymphadenopathy. However, the clinical presentation of patients with multicentric CD (MCD) ranged from mild lymphadenopathy with B-symptoms (5/8, 62.5%) to intense inflammation, vascular leak syndrome (3/8, 37.5%), hepatosplenomegaly (3/8, 37.5%), organ insufficiency (3/8, 37.5%), and even death (2/8, 25.0%). Compared with UCD patients, patients with MCD had significantly lower levels of hemoglobin (104 (90,129) vs 137 (120,149), p=0.018) and plasma albumin (31.5 (27.0,37.0) vs 45.0 (40.0,46.5), p=0.001), but IgG levels were significantly increased. Patients with UCD were mainly treated with surgical resection alone, with a five-year survival rate of 95.65%. When siltuximab is not an option, steroid plus rituximab-based chemotherapy and specific supportive care are common options for MCD. Except for 2 deaths, the remaining MCD patients have stable disease or partial remission. CONCLUSION: CD describes a heterogeneous group of disorders characterized by morphologically benign lymphoid hyperplasia. Notably, patients with MCD present varying degrees of inflammation responses, even involving multiple systems. Surgery is a direct and effective way to diagnose and treat UCD. In the absence of IL-6 antagonists, anti-inflammatory and immunosuppressive therapeutic strategies, and cytotoxic clearance of cells responsible for hypercytokinemia could be adopted.