Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantari Nagar, Pondicherry, India.
Int J Hematol. 2012 Jul;96(1):122-4. doi: 10.1007/s12185-012-1089-7. Epub 2012 May 3.
A nine-year-old girl presented with headache, purpura and mild left hemiparesis. Laboratory evaluation revealed thrombotic microangiopathy with ADAMTS13 deficiency, with auto-antibodies to ADAMTS13. She was treated with plasma exchange and steroids, following which she improved transiently, relapsing within 2 months. The relapse was refractory to conventional therapy and rituximab was tried. She had good response to rituximab and has been in remission for the past 12 months. Rituximab may be a promising option for children with acquired TTP refractory to plasma exchange and steroids.
一位 9 岁女孩因头痛、紫癜和轻度左侧偏瘫就诊。实验室评估显示存在血栓性微血管病和 ADAMTS13 缺乏症,伴有 ADAMTS13 自身抗体。她接受了血浆置换和类固醇治疗,治疗后短暂改善,但在 2 个月内复发。复发对常规治疗无效,尝试了利妥昔单抗。她对利妥昔单抗反应良好,过去 12 个月一直处于缓解状态。利妥昔单抗可能是对血浆置换和类固醇治疗无效的获得性 TTP 儿童的一种有前途的选择。