对依库珠单抗补体抑制有反应的严重难治性特发性温抗体型自身免疫性溶血性贫血
Severe refractory idiopathic warm autoimmune haemolytic anaemia responsive to complement inhibition with eculizumab.
作者信息
Neave Lucy, Wilson Andrew J, Lissack Maxine, Scully Marie
机构信息
Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK.
Department of Haematology, Barnet Hospital, Royal Free London NHS Foundation Trust, Hertfordshire, UK.
出版信息
BMJ Case Rep. 2018 Dec 13;11(1):e226429. doi: 10.1136/bcr-2018-226429.
Abstract
We report a case of severe idiopathic warm autoimmune haemolytic anaemia (wAIHA) which was initially poorly responsive to treatment with corticosteroids, intravenous immunoglobulin, mycophenolate mofetil and rituximab, and required transfusion of more than 30 units of red cells over 12 weeks. Off-label use of the terminal complement pathway inhibitor, eculizumab, led to rapid amelioration of the haemolysis, presumably by the inhibition of an intravascular component, and allowed time for slower acting immunosuppressive agent to take effect. This novel approach warrants further evaluation, given the poor prognosis of multirefractory wAIHA.
摘要
我们报告了一例严重的特发性温抗体型自身免疫性溶血性贫血（wAIHA）病例，该病例最初对皮质类固醇、静脉注射免疫球蛋白、霉酚酸酯和利妥昔单抗治疗反应不佳，在12周内需要输注超过30单位的红细胞。终末补体途径抑制剂依库珠单抗的超说明书使用导致溶血迅速改善，推测是通过抑制血管内成分实现的，并为起效较慢的免疫抑制剂发挥作用争取了时间。鉴于多难治性wAIHA预后较差，这种新方法值得进一步评估。
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