Castleman disease (CD) is a rare lymphoproliferative disease characterized by diverse clinical and pathologic features. Due to its rarity, there are limited studies comparing currently available therapies. The role of autologous stem cell transplantation (ASCT) in CD has not yet been established. In this paper, we describe the clinical characteristics, treatment choices, and outcomes in 34 Mayo Clinic patients diagnosed with multicentric CD from July 1, 2003 to April 30, 2018. Eighteen patients (53%) also met the criteria for POEMS, including 14 with the osteosclerotic variant. The first-line treatments included: steroid monotherapy (4), cytotoxic chemotherapy (6), rituximab alone (8) or with chemotherapy (2), anti-IL6 treatment (3), and ASCT (10). The median follow-up was 4.8 (range: 0.1-15.2) years. The 5-and 10-year overall survival rates were 84% and 71%, respectively. Sixteen patients received high-dose chemotherapy followed by ASCT during their disease course. Among those, 14 had multicentric CD associated with POEMS. There were no transplant-related deaths. All patients had at least a partial response to ASCT, most of whom achieved a complete response. The favorable outcomes seen with ASCT in this cohort suggest that transplantation may have a role in multicentric CD, particularly for patients with multicentric CD associated with POEMS.
| INTRODUCTIONCastleman disease (CD) is a rare lymphoproliferative disease driven by the overproduction of inflammatory cytokines, causing proliferation of benign lymphocytes. Since its initial description in 1954, 1 CD has been recognized as a spectrum of diseases with diverse clinical and pathologic features. [2][3][4][5] The localized unicentric form is usually asymptomatic or associated with mild symptoms. 3 However, multicentric Castleman disease (MCD), characterized by involvement of multiple lymph nodes, is a heterogeneous disease; patients present with symptoms of varying severities, cytopenias, autoimmune phenomena, and/or organ dysfunction. MCD is subdivided into two groups based on infection with the human herpes-virus 8 (HHV-8): HHV-8 positive and HHV-8 negative MCD, also known as idiopathic MCD (iMCD). [6][7][8] Clinical and laboratory features of MCD are mediated mainly by the inflammatory cytokine interleukin-6 (IL-6), thus providing a rationale for the use of IL-6 targeting monoclonal antibodies. In HHV-8 positive MCD, viral IL-6 is produced by HHV-8+ infected B cells, which may