[Lanadelumab在一名墨西哥遗传性I型血管性水肿患者中的初步经验]。

Carolina García-Rosas, J Jesús López-Tiro, Angélica Contreras-Contreras, Martha Ruiz-Peñaloza, Zayra Estefanía Ortiz-Monteón
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引用次数: 0

摘要

背景:遗传性1型血管性水肿(HAE1)是一种常染色体显性遗传疾病,其特征是C1抑制剂的定量和定性缺乏,缓激肽的过量产生,并导致不同严重程度的复发性血管性水肿,影响生活质量和生活本身。来自患者。Lanadelumab是一种人单克隆抗体,是血浆激肽释放酶的特异性抑制剂,被批准用于长期预防HAE1。病例报告:一名59岁的女性患者,自1987年11月以来被诊断为HAE1,对达那唑、新鲜冷冻血浆或静脉血浆来源的C1抑制剂没有治疗反应,由于血管水肿,需要每月3至9瓶醋酸艾替班。其具有高度改变的生活质量指数。开始每14天皮下注射300 mg Lanadelumab。在治疗开始时,AECT1评分为1分;AE-Qol2:57分,AAS3:32分,随访5、10、12个月。治疗一年后,记录显示AECT1为19分;AE-Qol2:36分,AAS3:5分。阿替班特醋酸酯的需求量每月不超过3瓶。结论:根据文献,拉纳德单抗显著降低了血管性水肿的活性,并对患者的生活质量产生了显著的积极影响,证实了拉纳德单抗是长期预防HAE的有效选择。
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[Experiencia inicial de Lanadelumab en una paciente mexicana con angioedema hereditario tipo I].

Background: Hereditary angioedema type 1 (HAE1) is an autosomal dominant disorder, characterized by quantitative and qualitative deficiency of C1 inhibitor, excessive production of bradykinin and causing recurrent angioedema in varying degrees of severity that affects quality of life and life itself. from the patients. Lanadelumab is a human monoclonal antibody, a specific inhibitor of plasma kallikrein, approved for long-term prophylaxis of HAE1.

Case report: A 59-year-old female patient, diagnosed with HAE 1 since November 1987, without therapeutic response to danazol, fresh frozen plasma, or C1 inhibitor derived from intravenous plasma, requiring 3 to 9 monthly vials of icatibant acetate due to angioedema. laryngeal, cutaneous and visceral with highly altered quality of life indices. Lanadelumab 300 mg subcutaneously every 14 days was started. At the start of treatment, the AECT1 score was 1 point; AE-Qol2: 57 points, AAS3: 32 points, being followed up at 5, 10 and 12 months. After one year of treatment, the records showed an AECT1 of 19 points; AE-Qol2: 36 points and AAS3: 5 points. The requirement for icatibant acetate has been no more than 3 vials per month.

Conclusion: In accordance with the literature, lanadelumab offered a significant decrease in angioedema activity and a significantly positive impact on the pa- tient's quality of life, confirming that lanadelumab is an effective option for long-term HAE prophylaxis. .

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