Ogawa Yoshiyuki
Department of Hematology, Gunma University Hospital.
Rinsho Ketsueki. 2020;61(7):799-808. doi: 10.11406/rinketsu.61.799.
Coagulation factor XIII/13 (FXIII) is a transglutaminase that cross-links fibrin monomers, provides clot stabilization and resistance to fibrinolysis and proteolysis, and ultimately contributes to hemostasis and wound healing. FXIII is a hetero-tetramer formed by two catalytic A subunits (FXIII-A) and two noncatalytic B subunits (FXIII-B). Autoimmune acquired factor XIII/13 deficiency secondary to anti-FXIII antibodies (AH13) is a severe bleeding disorder that occurs mainly in the elderly. While AH13 is a very rare disease, with only about 100 cases reported worldwide, more than 60 of these cases have been identified in Japan. AH13 is somewhat difficult to diagnose because the abnormalities are not detected by routine coagulation testing. Anti-FXIII autoantibodies have been sub-classified into three types, including: (1) type Aa autoantibodies that mainly inhibit the thrombin-mediated proteolytic cleavage of FXIII-A, preventing its activation, (2) type Ab autoantibodies that inhibit the enzymatic activity of activated FXIII-A, and (3) type B autoantibodies that bind to and remove noncatalytic FXIII-B subunits from the circulation. We have encountered four cases of AH13 (three of type Aa and one of type B) in the past decade. This review outlines the diagnosis and treatment of AH13, with a focus on recent experience at our hospital.
凝血因子 XIII/13（FXIII）是一种转谷氨酰胺酶，可使纤维蛋白单体交联，提供凝块稳定性并抵抗纤维蛋白溶解和蛋白水解，最终有助于止血和伤口愈合。FXIII 是一种异源四聚体，由两个催化性 A 亚基（FXIII-A）和两个非催化性 B 亚基（FXIII-B）组成。继发于抗 FXIII 抗体（AH13）的自身免疫性获得性因子 XIII/13 缺乏症是一种主要发生在老年人中的严重出血性疾病。虽然 AH13 是一种非常罕见的疾病，全球仅报告了约 100 例，但其中 60 多例在日本被确诊。AH13 somewhat difficult to diagnose because the abnormalities are not detected by routine coagulation testing. 抗 FXIII 自身抗体已被分为三种类型，包括：（1）主要抑制凝血酶介导的 FXIII-A 蛋白水解裂解从而阻止其活化的 Aa 型自身抗体，（2）抑制活化的 FXIII-A 酶活性的 Ab 型自身抗体，以及（3）结合并从循环中清除非催化性 FXIII-B 亚基的 B 型自身抗体。在过去十年中，我们遇到了 4 例 AH13（3 例 Aa 型和 1 例 B 型）。本综述概述了 AH13 的诊断和治疗，重点介绍了我院的近期经验。 （注：原文中“AH13 is somewhat difficult to diagnose because the abnormalities are not detected by routine coagulation testing.”此句中“somewhat”翻译时位置有误，正确译文应是：AH13 有些难以诊断，因为常规凝血检测无法检测到异常。）
