获得性血友病的管理:对阿拉伯联合酋长国从业人员的共识建议

Q4 Medicine
M. Marashi, A. Basha, A. Lal, H. Osman, Hasan Ghazal, S. Hashmi, M. Khanani
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引用次数: 0

摘要

获得性血友病A (AHA)是一种罕见的自身免疫性疾病,其特征是针对凝血因子VIII (FVIII)的自发抗体形成。在阿拉伯联合酋长国(UAE), AHA的治疗模式各不相同,导致患者预后不同。由于AHA病例通常呈现给缺乏疾病经验的临床从业人员,因此诊断有时会延迟。本文档旨在为阿联酋临床从业者提供建议,以弥合AHA临床管理中异质实践的差距。采用三阶段德尔菲法在PubMed和b谷歌Scholar中进行结构化文献检索。有证据表明,手术前激活部分凝血活酶时间延长或伴有急性或近期出血是AHA的标志。对于出血的AHA患者,建议使用旁路药物进行治疗。皮质类固醇和利妥昔单抗联合(不含)细胞毒性药物是FVIII患者的一线治疗方案:C <1 IU/dL。二线治疗包括第一轮治疗中未使用的药物。如果FVIII: C恢复到正常水平,建议进行血栓预防。这些建议旨在提高阿联酋血液学家和非血液学家对这种疾病的临床实践和认识,并提供实用的诊断和治疗建议。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Management of acquired hemophilia: Consensus recommendations for the United Arab Emirates practitioners
Acquired hemophilia A (AHA) is a rare autoimmune condition characterized by spontaneous antibodies formation against coagulation factor VIII (FVIII). In the United Arab Emirates (UAE), the treatment paradigm of AHA varies, leading to heterogeneous patient outcomes. Because AHA cases often present to clinical practitioners with insufficient experience in the disease, diagnosis is sometimes delayed. This document aims to provide recommendations to clinical practitioners in the UAE to bridge the gap of heterogeneous practice in the clinical management of AHA. Structured literature searches in PubMed and Google Scholar with a three-stage Delphi method were conducted. Evidence suggests that prolonged activated partial thromboplastin time before surgery or coupled with acute or recent onset of bleeding is a hallmark of AHA. Treatment using bypassing agents is recommended in AHA patients with bleeding. Corticosteroids and rituximab with (out) a cytotoxic agent represent the first-line treatment in patients with FVIII: C <1 IU/dL. Second-line therapy involves agents not utilized in the first round of treatment. Thromboprophylaxis is recommended if FVIII: C has returned to normal levels. These recommendations are intended to improve the clinical practice and awareness of this disorder among hematologists and nonhematologists in the UAE and provide practical diagnosis and treatment advice.
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来源期刊
Journal of Applied Hematology
Journal of Applied Hematology Medicine-Hematology
CiteScore
0.40
自引率
0.00%
发文量
34
审稿时长
24 weeks
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