Auto‐ and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences
Auto‐ and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences
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抗因子 XIII 的自身抗体和同种抗体：实验室诊断和临床后果
DOI：
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发表时间：
2018
影响因子：
10.4
通讯作者：
É. Katona
中科院分区：
文献类型：
--
作者：
L. Muszbek;K. Pénzes;É. Katona
获得性FXIII缺陷是由抗FXIII亚单位自身抗体引起的罕见但非常严重的出血疾病。FXIII缺陷患者的同种抗体也会导致危及生命的出血并发症，但这种情况极少发生。在这篇综述中，我们概述了抗FXIII抗体的诊断和分类，并分析了48例自身免疫性FXIII缺陷患者和另外4例FXIII缺陷患者产生抗FXIII同种抗体。这些患者是从同行评议的出版物中收集的，可以从中提取相关数据。除2例是针对FXIII-A的抗体外，本文还讨论了抗FXIII抗体存在时诊断FXIII缺乏症的困难，并推荐了抗FXIII抗体的功能分类方案。三个主要类别是中和抗体和非中和抗体以及具有联合作用的抗体。综述了目前用于检测和定量抑制FXIII激活和激活的FXIII转谷氨酰胺酶活性的方法，并概述了中和抗FXIII抗体的分类技术。强调了在这些情况下进行清关研究的重要性。结合试验可用于鉴定非中和抗体和结合型抗体，并通过实例证明了它们的信息力。在抗FXIII抗体阳性的患者中，最常见的出血症状是软组织出血；颅内出血也会发生，但频率低于遗传性FXIII缺乏症患者。这类患者的治疗极具挑战性；主要目标应该是根除抗体。
Acquired FXIII deficiencies caused by autoantibodies against FXIII subunits represent rare but very severe bleeding diatheses. Alloantibodies in FXIII‐deficient patients also cause life‐threatening bleeding complications, but they develop extremely rarely. In this review we provide an overview of the diagnosis and classification of anti‐FXIII antibodies and analyze 48 patients with autoimmune FXIII deficiency and four additional FXIII‐deficient patients who developed anti‐FXIII alloantibody. The patients were collected from peer‐reviewed publications from which relevant data could be extracted. With the exception of two cases the antibodies were directed against FXIII‐A. The difficulties in the diagnosis of FXIII deficiency in the presence of anti‐FXIII antibodies are discussed and a scheme for the functional classification of the anti‐FXIII antibodies is recommended. The three main categories are neutralizing and non‐neutralizing antibodies and antibodies with combined effect. The methods being used for detecting and quantifying the inhibitory effect on FXIII activation and on the transglutaminase activity of activated FXIII are summarized and techniques for the classification of neutralizing anti‐FXIII antibodies are outlined. The importance of clearance studies in these cases is emphasized. Binding assays, useful for the identification of non‐neutralizing and combined type antibodies, were collected from the literature and their informative power is demonstrated by examples. The most frequently occurring bleeding symptoms in patients with anti‐FXIII antibodies were soft tissue bleeding; intracranial bleedings also occurred, but less frequently than in inherited FXIII deficiency. Treatment of such patients is extremely challenging; the main aim should be eradication of the antibody.
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一种独特的 XIII 因子抑制剂，针对 XIIIA 因子上的纤维蛋白结合位点。
影响因子：
20.3
作者：
Fukue,H;Anderson,K;McPhedran,P;Clyne,L;McDonagh,J
通讯作者：
McDonagh,J
针对纤维蛋白稳定因子（因子 XIII）系统的自身免疫抗体（IgG Kansas）。
DOI：
10.1073/pnas.85.1.232
发表时间：
1988
影响因子：
11.1
作者：
Lorand,L;Velasco,PT;Rinne,JR;Amare,M;Miller,LK;Zucker,ML
通讯作者：
Zucker,ML
凝血因子 XIII 的 b 亚基完全缺乏的患者存在两种遗传缺陷。
影响因子：
20.3
作者：
Hashiguchi,T;Saito,M;Morishita,E;Matsuda,T;Ichinose,A
通讯作者：
Ichinose,A
出血患者体内的自身免疫抗体以独特的方式与凝血酶激活因子 XIII 相互作用。
影响因子：
20.3
作者：
Lorand,L;Velasco,PT;Murthy,SN;Lefebvre,P;Green,D
通讯作者：
Green,D