嗜血性关节病:如何早期诊断亚临床出血以及如何对受损关节进行骨科治疗。

IF 2.3 4区 医学 Q2 HEMATOLOGY Expert Review of Hematology Pub Date : 2023-07-01 Epub Date: 2023-07-04 DOI:10.1080/17474086.2023.2232547
E Carlos Rodriguez-Merchan
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引用次数: 0

摘要

引言:了解发达国家血友病性关节病的诊断、治疗、并发症和结果的现状很重要。涵盖领域:在PubMed上搜索2019年1月1日至2023年6月12日发表的文章。专家意见:在拥有专门血友病治疗中心的发达国家,初级血液学预防(2岁前开始 几年和不超过一次关节出血后)几乎完全消除了该疾病的关节相关问题。只有通过高强度和高剂量的预防才能实现零血红的理想目标:静脉输注凝血因子-标准半衰期或延长半衰期;定期或皮下注射非杀伤性产品(emicizumab或fitusiran)。然而,由于亚临床关节出血,亲血性关节病仍在发生。在一项研究中,16%未报告血栓形成的关节显示出先前亚临床出血的迹象(磁共振成像显示含/不含滑膜肥大的含铁血黄素沉积被视为先前亚临床流血的迹象),这为终身预防治疗的严重血友病患者的亚临床出血提供了证据。亚临床关节出血只有通过采取准确和有针对性的预防措施才能避免。
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Hemophilic arthropathy: how to diagnose subclinical bleeding early and how to orthopedically treat a damaged joint.

Introduction: It is important to know the current status of hemophilic arthropathy diagnoses, treatments, complications, and outcomes in developed countries.

Areas covered: A bibliographic search in PubMed for articles published from 1 January 2019 through 12 June 2023 was performed.

Expert opinion: In developed countries with specialized hemophilia treatment centers, primary hematological prophylaxis (started before the age of 2 years and after no more than one joint bleed) has almost completely eliminated the joint-related problems of the disease. The ideal goal of zero hemarthroses can be achieved only with intense and well-dosed prophylaxis: intravenous infusion of coagulation factor - standard half-life or extended half-life; periodic or subcutaneous injections of nonfactor products (emicizumab or fitusiran). However, hemophilic arthropathy continues to occur due to subclinical joint hemorrhages. In one study, 16% of the joints without reported hemarthroses showed signs of previous subclinical bleeding (hemosiderin deposits with/without synovial hypertrophy on magnetic resonance imaging were deemed signs of previous subclinical bleeding), rendering evidence for subclinical bleeding in people with severe hemophilia with lifelong prophylaxis treatment. Subclinical joint hemorrhages can be averted only by employing accurate and tailored prophylaxis.

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来源期刊
CiteScore
4.70
自引率
3.60%
发文量
98
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.
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