siltuximab:首个治疗多中心型Castleman’s病孤儿药

Siltuximab: the first orphan drug for treatment of multicentric Castleman's disease

多中心型Castleman’s病(MCD)是由于某种类型的白细胞过度生产导致淋巴结肿大,该病的发病机制不明确,可能与淋巴结中活化B细胞内白细胞介素-6(IL-6)的失调或不平衡过量生产有关,临床较为少见。siltuximab通过靶向IL-6发挥作用。临床上siltuximab的推荐剂量为11 mg·kg-1,静脉滴注,每3周1次。各项研究表明该药对HIV阴性、HHV-8阴性的MCD患者安全、有效。

Muhicentric Castleman＇ s disease （MCD） is a rare disorder in which lymphocytes, a certain type of white blood cells, are over-produced and lead to enlargement of lymph nodes, and pathogenesis of the disease is not yet clear, dysregulated, or imbalanced, overproduction of IL-6 from activated B cells in affected lymph nodes has been implicated in MCD, clinical relatively rare. Siltuximab 11 mg·kg^-1 is given as an intravenous infusion administered every 3 weeks. Siltuximab is security and effective for the treatment of patients with MCD who are human immunodeficiency virus （HIV） negative and human herpesvirus- 8 （HHV- 8） negative in clinical trail.