Division of Pediatric Hematology/Oncology, University of Minnesota, Minneapolis, Minnesota.
Pediatr Blood Cancer. 2014 Apr;61(4):737-9. doi: 10.1002/pbc.24761. Epub 2013 Sep 9.
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.
血管滤泡性淋巴结增生症是一种罕见的淋巴组织增生性疾病，可表现为局灶性或多中心性。多中心血管滤泡性淋巴结增生症（MCD）常伴有明显的全身症状，部分与疾病发病机制中白细胞介素 6 的潜在作用有关。MCD 的治疗尚未得到充分确立，其预后历来较差。我们报告了一例严重的儿童多中心血管滤泡性淋巴结增生症病例，该患者在接受多药物化疗和白细胞介素 6 受体抑制剂托珠单抗靶向维持治疗后，已获得持续缓解。这是首例在停止托珠单抗治疗后，儿童患者的多中心血管滤泡性淋巴结增生症获得持续缓解的病例报告。
