Inhibitor eradication and treatment for acquired hemophilia A.

IF 2.3 4区医学 Q2 HEMATOLOGY

Expert Review of Hematology Pub Date : 2024-06-01 Epub Date: 2024-05-09 DOI:10.1080/17474086.2024.2352505

Massimo Franchini, Daniele Focosi

{"title":"Inhibitor eradication and treatment for acquired hemophilia A.","authors":"Massimo Franchini, Daniele Focosi","doi":"10.1080/17474086.2024.2352505","DOIUrl":null,"url":null,"abstract":"Introduction: Acquired hemophilia A (AHA) is a rare hemorrhagic autoimmune disorder characterized by autoantibodies against coagulation factor VIII (FVIII). In approximately half of the cases AHA does not recognize any cause (idiopathic form), while in the other cases it may be triggered by autoimmune disorders, cancers, drugs, infections, or pregnancy. Besides treating the underlying disorder, specific AHA treatment includes management of bleeding, if necessary, and inhibitor eradication.Areas covered: This narrative review summarizes the main epidemiological, clinical, laboratory, and therapeutic characteristics of AHA. In particular, it is focused on the current therapeutic options for the inhibitor eradication, also showing the latest findings on the innovative therapies. A literature search strategy was performed, without temporal limits, through Medline and PubMed electronic databases.Expert opinion: Various first-line and second-line immunosuppressive agents are currently available for the management of AHA. Among the latter, the anti-CD20 monoclonal antibody rituximab has been the object of intense research during the last years from investigators as innovative promising eradicating therapy for AHA. Preliminary data from the studies support the use of this drug as a first-line option for newly diagnosed AHA cases.","PeriodicalId":12325,"journal":{"name":"Expert Review of Hematology","volume":" ","pages":"233-240"},"PeriodicalIF":2.3000,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Expert Review of Hematology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1080/17474086.2024.2352505","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/5/9 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"HEMATOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Introduction: Acquired hemophilia A (AHA) is a rare hemorrhagic autoimmune disorder characterized by autoantibodies against coagulation factor VIII (FVIII). In approximately half of the cases AHA does not recognize any cause (idiopathic form), while in the other cases it may be triggered by autoimmune disorders, cancers, drugs, infections, or pregnancy. Besides treating the underlying disorder, specific AHA treatment includes management of bleeding, if necessary, and inhibitor eradication.

Areas covered: This narrative review summarizes the main epidemiological, clinical, laboratory, and therapeutic characteristics of AHA. In particular, it is focused on the current therapeutic options for the inhibitor eradication, also showing the latest findings on the innovative therapies. A literature search strategy was performed, without temporal limits, through Medline and PubMed electronic databases.

Expert opinion: Various first-line and second-line immunosuppressive agents are currently available for the management of AHA. Among the latter, the anti-CD20 monoclonal antibody rituximab has been the object of intense research during the last years from investigators as innovative promising eradicating therapy for AHA. Preliminary data from the studies support the use of this drug as a first-line option for newly diagnosed AHA cases.

查看原文本刊更多论文

后天性 A 型血友病的抑制剂根除和治疗。

导言：获得性血友病 A（AHA）是一种罕见的出血性自身免疫性疾病，其特征是针对凝血因子 VIII（FVIII）的自身抗体。约有一半的 AHA 病例没有任何病因（特发性），而其他病例则可能由自身免疫性疾病、癌症、药物、感染或妊娠引发。除了治疗潜在的疾病外，AHA 的具体治疗方法还包括必要时控制出血和消除抑制剂：这篇叙述性综述总结了 AHA 的主要流行病学、临床、实验室和治疗特点。本综述总结了 AHA 的主要流行病学、临床、实验室和治疗特点，尤其关注当前根除抑制剂的治疗方案，并展示了创新疗法的最新发现。通过 Medline 和 PubMed 电子数据库执行了无时间限制的文献检索策略：目前有多种一线和二线免疫抑制剂可用于治疗 AHA。在后者中，抗 CD20 单克隆抗体利妥昔单抗是研究人员在过去几年中进行深入研究的对象，认为它是有希望根除 AHA 的创新疗法。研究的初步数据支持将这种药物作为新诊断的 AHA 病例的一线选择。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Expert Review of Hematology HEMATOLOGY-

CiteScore

4.70

自引率

3.60%

发文量

审稿时长

6-12 weeks

期刊介绍： Advanced molecular research techniques have transformed hematology in recent years. With improved understanding of hematologic diseases, we now have the opportunity to research and evaluate new biological therapies, new drugs and drug combinations, new treatment schedules and novel approaches including stem cell transplantation. We can also expect proteomics, molecular genetics and biomarker research to facilitate new diagnostic approaches and the identification of appropriate therapies. Further advances in our knowledge regarding the formation and function of blood cells and blood-forming tissues should ensue, and it will be a major challenge for hematologists to adopt these new paradigms and develop integrated strategies to define the best possible patient care. Expert Review of Hematology (1747-4086) puts these advances in context and explores how they will translate directly into clinical practice.