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

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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.

Original languageEnglish (US)
Pages (from-to)737-739
Number of pages3
JournalPediatric Blood and Cancer
Issue number4
StatePublished - Apr 1 2014



  • Anakinra
  • CHOP
  • Multicentric Castleman disease
  • Tocilizumab

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