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评估康西珠单抗在甲型/乙型血友病患者中的安全性和有效性:一项系统评价。

Evaluating the Safety and Efficacy of Concizumab in Hemophilia A/B Patients: A Systematic Review.

作者信息

Siddiqui Erum, Khalid Maliha, Khan Muhammad Saad, Farhan Kanza, Khan Muhammad Mohsin, Waafira Aminath

机构信息

Department of Medicine, Jinnah Sindh Medical University, Karachi, Pakistan.

School of Medicine, The Maldives National University, Malé, Maldives.

出版信息

Clin Appl Thromb Hemost. 2025 Jan-Dec;31:10760296251342968. doi: 10.1177/10760296251342968. Epub 2025 May 14.

DOI:10.1177/10760296251342968
PMID:40368339
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12078972/
Abstract

BackgroundHemophilia A and B are X-linked recessive bleeding disorders caused by deficiencies of coagulation factors VIII and IX, respectively. These conditions lead to spontaneous bleeding, joint damage, inhibitor development, and the burden of frequent intravenous infusions. Concizumab, a monoclonal antibody targeting tissue factor pathway inhibitor (TFPI), is a novel non-factor therapy that enhances thrombin generation. This systematic review evaluates the efficacy and safety of concizumab prophylaxis in patients with hemophilia A and B.MethodsThis systematic review was conducted in accordance with PRISMA guidelines. Randomized controlled trials (RCTs) assessing the use of concizumab in hemophilia A or B were identified through a comprehensive search of electronic databases. Outcomes of interest included annualized bleeding rate (ABR), thrombin generation, bleeding episodes, immunogenicity, and adverse events. The Cochrane Risk of Bias Tool 2.0 was used for quality assessment.ResultsFive studies were included. Concizumab prophylaxis was associated with a notable reduction in ABR, with reported decreases from 9.4 to 1.3 episodes/year and from 19.6 to 2.9 episodes/year in hemophilia A, and from 14.9 to 1.6 episodes/year in hemophilia B. Thrombin generation increased in a dose-dependent manner and stabilized by week 24. Across all studies, bleeding episodes were significantly reduced. Adverse events were primarily mild to moderate. No thromboembolic events were reported.ConclusionConcizumab appears to be an effective and safe prophylactic treatment for patients with hemophilia A and B, demonstrating consistent reductions in bleeding rates and enhanced thrombin generation. Further long-term studies are warranted to establish its sustained safety and efficacy.

摘要

背景

甲型和乙型血友病是分别由凝血因子VIII和IX缺乏引起的X连锁隐性出血性疾病。这些病症会导致自发性出血、关节损伤、抑制剂形成以及频繁静脉输注的负担。康西珠单抗是一种靶向组织因子途径抑制剂(TFPI)的单克隆抗体,是一种新型的非因子疗法,可增强凝血酶生成。本系统评价评估了康西珠单抗预防甲型和乙型血友病患者的疗效和安全性。

方法

本系统评价按照PRISMA指南进行。通过全面检索电子数据库,识别评估康西珠单抗在甲型或乙型血友病中应用的随机对照试验(RCT)。感兴趣的结局包括年化出血率(ABR)、凝血酶生成、出血发作、免疫原性和不良事件。使用Cochrane偏倚风险工具2.0进行质量评估。

结果

纳入了五项研究。康西珠单抗预防与ABR显著降低相关,在甲型血友病中报告的年发作次数从9.4次降至1.3次,从19.6次降至2.9次,在乙型血友病中从14.9次降至1.6次。凝血酶生成呈剂量依赖性增加,并在第24周时稳定。在所有研究中,出血发作均显著减少。不良事件主要为轻度至中度。未报告血栓栓塞事件。

结论

康西珠单抗似乎是甲型和乙型血友病患者一种有效且安全的预防性治疗方法,出血率持续降低,凝血酶生成增强。需要进一步进行长期研究以确定其持续的安全性和疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/b44f93ebb238/10.1177_10760296251342968-fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/797dca280923/10.1177_10760296251342968-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/9230f246f29c/10.1177_10760296251342968-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/965b99e06ffe/10.1177_10760296251342968-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/a55dd3343243/10.1177_10760296251342968-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/b44f93ebb238/10.1177_10760296251342968-fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/797dca280923/10.1177_10760296251342968-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/9230f246f29c/10.1177_10760296251342968-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/965b99e06ffe/10.1177_10760296251342968-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/a55dd3343243/10.1177_10760296251342968-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/335c/12078972/b44f93ebb238/10.1177_10760296251342968-fig5.jpg

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本文引用的文献

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Lancet Haematol. 2024 Dec;11(12):e891-e904. doi: 10.1016/S2352-3026(24)00307-7. Epub 2024 Nov 6.
2
Concizumab improves clot formation in hemophilia A under flow.康西珠单抗可改善A型血友病在血流状态下的凝血形成。
J Thromb Haemost. 2024 Sep;22(9):2438-2448. doi: 10.1016/j.jtha.2024.05.020. Epub 2024 May 28.
3
Non-clotting factor therapies for preventing bleeds in people with congenital hemophilia A or B.
用于预防先天性 A 型或 B 型血友病患者出血的非凝血因子治疗方法。
Cochrane Database Syst Rev. 2024 Feb 27;2(2):CD014544. doi: 10.1002/14651858.CD014544.pub2.
4
Phase 3 Trial of Concizumab in Hemophilia with Inhibitors.康西珠单抗治疗伴有抑制剂的血友病的 3 期临床试验。
N Engl J Med. 2023 Aug 31;389(9):783-794. doi: 10.1056/NEJMoa2216455.
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Clinical pharmacology of emicizumab for the treatment of hemophilia A.用于治疗A型血友病的艾美赛珠单抗的临床药理学
Expert Rev Clin Pharmacol. 2023 Jul-Dec;16(9):775-790. doi: 10.1080/17512433.2023.2243213. Epub 2023 Aug 21.
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Nonfactor Therapies for Hemophilia.血友病的非凝血因子治疗方法
Hemasphere. 2023 Jun 6;7(6):e911. doi: 10.1097/HS9.0000000000000911. eCollection 2023 Jun.
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Foundations of hemophilia and epidemiology.血友病与流行病学基础。
Blood Coagul Fibrinolysis. 2023 Jun 1;34(S1):S35-S36. doi: 10.1097/MBC.0000000000001222.
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The PRISMA 2020 statement: an updated guideline for reporting systematic reviews.PRISMA 2020 声明:系统评价报告的更新指南。
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