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

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.

Turcotte LM ，Correll CK ，Reed RC ，Moertel CL ... -  《-》

Sustained remission of multicentric Castleman disease in children treated with tocilizumab, an anti-interleukin-6 receptor antibody.

Galeotti C ，Boucheron A ，Guillaume S ，Koné-Paut I ... -  《-》

Short-term efficacy of the IL6 receptor antibody tocilizumab in patients with HIV-associated multicentric Castleman disease: report of two cases.

Nagao A ，Nakazawa S ，Hanabusa H 《Journal of Hematology & Oncology》

Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease?

Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. We present a case of a patient, negative for HIV and HHV-8, who had an unsuccessful surgical intervention and no response to radiotherapy and chemotherapy. He had severe paraneoplastic pemphigus and was treated with tocilizumab, an anti-interleukin-6 receptor monoclonal antibody that has demonstrated good response rates in multicentric Castleman disease but demonstrated no clinical response despite 2 months of treatment. Our report is the first to describe a lack of response to tocilizumab in the rare setting of refractory UCD and discuss potential for distinct disease biology.

Abid MB ，Peck R ，Abid MA ，Al-Sakkaf W ，Zhang Y ，Dunnill GS ，Staines K ，Sequeiros IM ，Lowry L ... -  《-》

Treatment of multicentric Castleman disease through combination of tocilizumab, lenalidomide and glucocorticoids: Case report.

Cai S ，Zhong Z ，Li X ，Wang HX ，Wang L ，Zhang M ... -  《-》