Multicentric Castleman’s disease in HIV patients: a single-center cohort diagnosed from 2008 to 2018

PURPOSE: Castleman’s disease (CD) is a well-established entity but there is a lack of available data regarding the management and therapy of HIV- and HHV-8-positive multicentric CD (MCD). We provide our own single-center experience with HIV-associated MCD. METHODS: We performed a retrospective, descriptive study on a cohort of patients with MCD, diagnosed and admitted to the infectious diseases or intensive care unit in the University Hospital Düsseldorf between 2008 and 2018. Included patients had a previous or new HIV diagnosis and clinical signs resembling MCD with evidence of HHV-8 replication or histological diagnosis for MCD. RESULTS: Nine male patients were included in the study. All patients were treated with Rituximab after diagnosis of MCD, with six of them acquiring resolution of symptoms. Three patients received tocilizumab additionally. Other treatment options included: splenectomy (2/9), valganciclovir (2/9), vincristine and siltuximab (1/9), ruxolitinib and Cytosorb(®) (2/9). The relapse rate was 44% (4/9) and the survival rate 87.5% after 1 year (8/9) and 71.4% after 3 years (5/7). CONCLUSION: The most effective first-line therapy and retreatment option remains rituximab. The effectiveness of other treatment options like splenectomy or different immunotherapeutic approaches requires confirmation in larger-scale studies.