Sustained remission of severe multicentric castleman disease following multiagent chemotherapy and tocilizumab maintenance

Abstract

Castleman disease is a rare lymphoproliferative disorder, which presents in a unicentric or multicentric fashion. Multicentric Castleman disease (MCD) is associated with significant systemic symptoms, in part related to the underlying role of interleukin‐6 in disease pathogenesis. Treatment for MCD has not been well established and prognosis has historically been poor. We present a case of severe MCD in a pediatric patient who has shown sustained remission following multi‐agent chemotherapy and targeted maintenance therapy with the interleukin‐6 receptor inhibitor, tocilizumab. This represents the first case report of sustained remission of MCD in a pediatric patient following discontinuation of tocilizumab therapy. Pediatr Blood Cancer