在一名C1抑制剂正常且基因检测呈阴性的遗传性血管性水肿患者中成功使用了lanadelumab。
Successful use of lanadelumab in a patient with hereditary angioedema with normal C1 inhibitor and negative genetic testing.
作者信息
Adatia Adil, Ritchie Bruce
机构信息
Division of Pulmonary Medicine, Department of Medicine, University of Alberta, Edmonton, Canada.
Division of Hematology, Department of Medicine, University of Alberta, Edmonton, Canada.
出版信息
J Allergy Clin Immunol Glob. 2023 Feb 21;2(2):100087. doi: 10.1016/j.jacig.2023.100087. eCollection 2023 May.
Abstract
We report an approximately 80% reduction in angioedema attacks with lanadelumab, a mAb targeting plasma kallikrein, in a case of hereditary angioedema with normal C1 inhibitor levels. This finding supports a central pathophysiologic role for kallikrein in hereditary angioedema with normal C1 levels and supports the need for prospective studies of lanadelumab use with this condition.
摘要
我们报告了在1例C1抑制剂水平正常的遗传性血管性水肿患者中，使用靶向血浆激肽释放酶的单克隆抗体lanadelumab后血管性水肿发作减少了约80%。这一发现支持了激肽释放酶在C1水平正常的遗传性血管性水肿中的核心病理生理作用，并支持对lanadelumab用于这种情况进行前瞻性研究的必要性。
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