获得的XIII因子缺乏症:一个范围审查.
Olivier Duranteau1, Guney Tatar1, Anne Demulder1
1From Anesthesiology Service Department, CUB-ULB Hôpital Erasme, Brussels, Belgium (OD, GT, TT), Intensive Care Unit, Percy Military Training Hospital, Clamart, France (OD), Faculté de Médecine, Université Libre de Bruxelles, Brussels, Belgium (OD, AD, TT), Laboratory of Hematology LHUB-ULB ULB, Université Libre de Bruxelles, Brussels, Belgium (AD).
European journal of anaesthesiology and intensive care
|February 7, 2025
概括
获得的XIII因子 (FXIII) 缺乏,通常是自身免疫性,呈现出出血症状. 治疗包括FXIII缩剂,免疫抑制剂如皮质类固醇或自身免疫病例的rituximab,或单独缩以消费为基础的缺乏症.
科学领域:
- 血液学 血液学 血液学
- 免疫学 免疫学 免疫学
- 内部医学 内部医学
背景情况:
- 获得的XIII因子 (FXIII) 缺乏症是一种罕见的静血性疾病.
- 它可以源于自身免疫抗体的产生或非免疫因素,如减少合成或增加消费.
- 这种情况带来了诊断和治疗的挑战.
研究的目的:
- 审查获得的FXIII缺乏症的临床表现,诊断方法和治疗策略.
- 确定最有效和最常用的治疗方法.
- 根据不同的治疗干预来分析患者的结果.
主要方法:
- 进行了系统的文献审查.
- 搜索的数据库包括PubMed,Medline和Embase/Ovid从2012年1月到2022年4月的时间.
- 使用的关键词是"获得的FXIII缺陷";36篇文章符合资格标准.
主要成果:
- 大多数获得的FXIII缺陷病例 (24/30) 是自身免疫的.
- 局部血液瘤是最常见的症状 (31例).
- 治疗包括FXIII缩剂,皮质类固醇,环胺或利图西马布用于自身免疫病例,以及FXIII缩剂用于基于消费的缺乏症.
结论:
- 获得的FXIII缺乏症需要根据其潜在原因 (自身免疫与非免疫) 进行量身定制的治疗.
- 有效的管理策略包括免疫抑制疗法和FXIII度补充剂.
- 迅速和适当的治疗对于改善患者的治疗结果和降低死亡率至关重要.
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