Amélie Launois, Isabelle Martin-Toutain, Floriane Devaux, Juliette Lambert, Thomas Longval, Fatiha Merabet, Rym Jaidi, Sophie Le Dore, Emmanuelle Ferre, Philippe Rousselot, Emmanuelle De Raucourt, Claire Flaujac
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引用次数: 0
Abstract
Emicizumab is a bispecific antibody that mimics the function of factor VIII (FVIII) and is indicated for prophylactic use in patients with congenital hemophilia A with or without inhibitors. Acquired hemophilia A (AHA) is a rare and severe disorder causes by autoantibodies that inhibit FVIII. In AHA, acute bleeding are managed with bypassing agents but several reports described the off-label use of emicizumab. The aim of this article is to describe two cases of AHA treated with emicizumab and a review of the scientific littérature. Reports indicate that the use of emicizumab is efficacious to treat acute bleeding with less thrombotic events thant with bypassing agents and with a reduced hospitalisation duration. Nevertheless biological monitoring is more complicated with assay interferences and a persistent circulation more than 6 months after the last injection was observed for our two patients.
Emicizumab 是一种模仿因子 VIII (FVIII) 功能的双特异性抗体，适用于有或没有抑制因子的先天性 A 型血友病患者的预防性治疗。获得性血友病 A（AHA）是一种罕见的严重疾病，由抑制 FVIII 的自身抗体引起。在 AHA 中，急性出血可通过旁路药物进行处理，但有几篇报道描述了标签外使用埃米珠单抗的情况。本文旨在描述两例使用埃米珠单抗治疗 AHA 的病例，并对科学文献进行回顾。报告显示，使用埃米珠单抗治疗急性出血疗效显著，与旁路药物相比血栓事件较少，住院时间缩短。然而，生物监测因检测干扰而变得更加复杂，我们的两名患者在最后一次注射后超过 6 个月仍出现持续循环。
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