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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.

SUBMITTER: Turcotte LM 

PROVIDER: S-EPMC3946294 | biostudies-literature | 2014 Apr

REPOSITORIES: biostudies-literature

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Sustained remission of severe Multicentric Castleman disease following multiagent chemotherapy and tocilizumab maintenance.

Turcotte Lucie M LM   Correll Colleen K CK   Reed Robyn C RC   Moertel Christopher L CL  

Pediatric blood & cancer 20130909 4


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  ...[more]

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