Duranteau Olivier, Tatar Guney, Demulder Anne, Tuna Turgay
From 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).
Eur J Anaesthesiol Intensive Care. 2023 Sep 29;2(5):e0035. doi: 10.1097/EA9.0000000000000035. eCollection 2023 Oct.
Acquired factor XIII (FXIII) deficiency is a very rare haemostatic defect that can be either immune (rare development of an autoantibody targeting FXIII epitopes) or nonimmune (diminished synthesis or increased consumption of the same factor). The aim of this study is to review the symptomatology, the diagnostic method used, but above all to determine the most frequently used and potentially most effective treatment for acquired FXIII deficiency. PubMed, Medline, embase/Ovid databases were queried from 1 January 2012 to 3 April 2022. Data extraction was performed using the keywords 'Acquired FXIII deficiency.' The systematic search identified 474 records. After screening titles and abstracts, only 36 articles met the eligibility criteria. The mean age of all patients was 57.6 [range, 1-84] years. The male to female ratio was 35 : 25. The majority of cases described were due an autoimmune reaction with antibody production (24 manuscripts), only six manuscripts described consumption. The most prevalent symptoms were local haematoma (31). Six cases died, two from haemorrhagic shock, two from haemorrhagic stroke, one from respiratory distress, and 1 from septic shock. Given the patient outcomes, this review confirms that the most appropriate treatment consists of one of the following elements or a combination of several of these elements: FXIII concentrate, corticosteroids, cyclophosphamide, rituximab for autoimmune cases, and FXIII concentrate supplementation only in case of consumption.
获得性因子 XIII（FXIII）缺乏症是一种非常罕见的止血缺陷，可分为免疫性（罕见的针对 FXIII 表位的自身抗体形成）或非免疫性（同一因子的合成减少或消耗增加）。本研究的目的是回顾其症状学、所采用的诊断方法，但最重要的是确定获得性 FXIII 缺乏症最常用且可能最有效的治疗方法。检索了 2012 年 1 月 1 日至 2022 年 4 月 3 日期间的 PubMed、Medline、embase/Ovid 数据库。使用关键词“获得性 FXIII 缺乏症”进行数据提取。系统检索共识别出 474 条记录。在筛选标题和摘要后，仅有 36 篇文章符合纳入标准。所有患者的平均年龄为 57.6 [范围 1 - 84] 岁。男女比例为 35∶25。所描述的大多数病例是由于自身免疫反应伴抗体产生（24 篇手稿），仅有 6 篇手稿描述了消耗情况。最常见的症状是局部血肿（31 例）。6 例患者死亡，2 例死于失血性休克，2 例死于出血性中风，1 例死于呼吸窘迫，1 例死于感染性休克。鉴于患者的结局，本综述证实最合适的治疗包括以下一种要素或几种要素的组合：FXIII 浓缩物、皮质类固醇、环磷酰胺、用于自身免疫性病例的利妥昔单抗，以及仅在消耗情况下补充 FXIII 浓缩物。
