Reduced difference of α-plasmin inhibitor levels between plasma and serum in patientswith severe factor XIII deficiency,including autoimmune hemorrhaphiliadue to anti-factor XIII antibodies
Reduced difference of α-plasmin inhibitor levels between plasma and serum in patientswith severe factor XIII deficiency,including autoimmune hemorrhaphiliadue to anti-factor XIII antibodies
复制标题
严重因子 XIII 缺乏症（包括抗因子 XIII 抗体导致的自身免疫性血友病）患者血浆和血清之间 α-纤溶酶抑制剂水平差异减小
DOI：
10.1007/s12185-011-0992-7
复制
发表时间：
2012
期刊：
影响因子：
2.1
通讯作者：
Souri M.
中科院分区：
文献类型：
--
作者：
Ichinose A;Souri M.
凝血因子XIII/13（FXIII/13）通过与其他纤维蛋白分子以及α2-纤溶酶抑制剂（α2-PI）形成交联来稳定纤维蛋白分子。“出血性获得性FXIII/13缺乏症”以前被认为是罕见的，但最近在日本有所增加。在10个月的全国性活动中，我们在检查了20例新的疑似“出血性获得性FXIII/13缺乏症”后，诊断出5例新的“抗FXIII/13自身抗体所致获得性血友病”患者。当FXIII/13活性降低至低于正常值的50%时，其与血浆和血清（血浆-血清α2-PI）之间α2-PI水平的差异成比例，可能是由于其通过活化的FXIII/13与纤维蛋白交联。因此，离体血浆-血清α2-PI含量降低可能反映了体内FXIII/13活性降低。因此，血浆-血清α2-PI也可能是诊断严重FXIII/13缺乏症的有用标志物。
Coagulation factor XIII/13 (FXIII/13) stabilizes fibrin molecules by creating crosslinks with other fibrin molecules as well as with α2-plasmin inhibitor (α2-PI). “Hemorrhagic acquired FXIII/13 deficiency” was formerly considered rare, but has been increasing recently in Japan. During the 10 months of our nationwide campaign, we diagnosed five new patients with “acquired hemorrhaphilia due to anti-FXIII/13 autoantibodies,” after examining 20 newly suspected cases of “hemorrhagic acquired FXIII/13 deficiency.” When FXIII/13 activity was reduced to less than 50% of normal, it was proportional to the difference in α2-PI levels between plasma and serum (plasma–serum α2-PI), likely due to its cross-linking to fibrin by activated FXIII/13. Accordingly, decreased amounts of the plasma–serum α2-PI ex vivo may reflect reduced FXIII/13 activity in vivo. The plasma–serum α2-PI may thus also be a useful diagnostic marker for severe FXIII/13 deficiency.