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一种获得性、钙依赖性、因子 X 抑制剂。

An acquired, calcium-dependent, factor X inhibitor.

机构信息

Division of Hematology, Washington University School of Medicine, St. Louis, MO 63110, USA.

出版信息

Blood Cells Mol Dis. 2014 Feb-Mar;52(2-3):116-20. doi: 10.1016/j.bcmd.2013.09.001. Epub 2013 Sep 26.

DOI:10.1016/j.bcmd.2013.09.001
PMID:24074948
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3947207/
Abstract

Acquired factor X (FX) deficiency unrelated to amyloidosis is a rare disorder in which an anti-FX antibody is infrequently detected. A patient with severe bleeding due to a calcium ion-dependent anti-FX IgG antibody is described. The FX affinity purified IgG bound the light chain of FX, but not FX lacking its γ-carboxyglutamic acid domain, and binding was enhanced >1000-fold in the presence of calcium ions. The antibody also recognized prothrombin and factor VII with about 100-fold and 1000-fold lower affinity. Like a lupus anticoagulant, increasing concentrations of phospholipids in functional assays reduced the inhibitory activity of the antibody. The effect of these properties of the inhibitor on laboratory diagnostic studies is considered.

摘要

获得性 FX（FX）因子缺乏与淀粉样变性无关，是一种罕见的疾病，很少检测到抗 FX 抗体。描述了一名因钙离子依赖性抗 FX IgG 抗体导致严重出血的患者。FX 亲和力纯化 IgG 结合 FX 的轻链，但不结合缺乏 γ-羧基谷氨酸结构域的 FX，在钙离子存在下结合增强>1000 倍。该抗体还识别凝血酶原和因子 VII，亲和力约低 100 倍和 1000 倍。与狼疮抗凝剂一样，功能测定中磷脂浓度的增加降低了抗体的抑制活性。考虑了抑制剂的这些特性对实验室诊断研究的影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/c78c1bf0b19f/nihms-528077-f0005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/d21965fd6644/nihms-528077-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/a3a7e81bb836/nihms-528077-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/2f4c5e69f5ec/nihms-528077-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/4af3dc8e8605/nihms-528077-f0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/c78c1bf0b19f/nihms-528077-f0005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/d21965fd6644/nihms-528077-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/a3a7e81bb836/nihms-528077-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/2f4c5e69f5ec/nihms-528077-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/4af3dc8e8605/nihms-528077-f0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3642/3947207/c78c1bf0b19f/nihms-528077-f0005.jpg

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本文引用的文献

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2
Factor X inhibitor: a fulminant presentation and fatal course of a rare syndrome in a 59-year-old male.X 因子抑制剂：59 岁男性罕见综合征的暴发性表现和致死过程。
Acta Haematol. 2013;129(1):40-4. doi: 10.1159/000342115. Epub 2012 Oct 26.
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Clinical review: Prothrombin complex concentrates--evaluation of safety and thrombogenicity.临床综述：凝血酶原复合物浓缩物——安全性和血栓形成性评估。
Crit Care. 2011;15(1):201. doi: 10.1186/cc9311. Epub 2011 Jan 12.
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The Kunitz-3 domain of TFPI-alpha is required for protein S-dependent enhancement of factor Xa inhibition.TFPI-α 的 Kunitz-3 结构域是蛋白 S 依赖性增强因子 Xa 抑制所必需的。
Blood. 2010 Aug 26;116(8):1344-51. doi: 10.1182/blood-2009-10-246686. Epub 2010 May 17.
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Lupus anticoagulant associated with transient severe factor X deficiency: a report of two patients presenting with major bleeding complications.狼疮抗凝物伴短暂性严重因子X缺乏：两例出现严重出血并发症患者的报告
Br J Haematol. 2003 May;121(4):639-42. doi: 10.1046/j.1365-2141.2003.04325.x.
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