Departments of Pediatric Hematology-Oncology.
Biochemistry.
J Pediatr Hematol Oncol. 2021 Jan;43(1):e99-e102. doi: 10.1097/MPH.0000000000001671.
Coagulation factor XIII (FXIII) is a fibrin-stabilizing factor with additional roles in wound healing and interactions between the decidua and fetus. Congenital FXIII deficiency is rare bleeding disorder. Inhibitor development against FXIII in inherited FXIII deficency is also uncommon, but may cause severe, life-threatening bleeding. FXIII is the last step in the coagulation cascade with normal coagulation paramaters (PT, aPTT), the detection of inhibitor to FXIII is quite difficult. The treatment of inhibitor-positive congenital FXIII deficiency is challenging due to the lack of a role of by-pass agents such as FVII. The best known ways of treatment in these cases are the use of high-dose FXIII concentrates and immunosuppression. Herein, we report the management of postoperative bleeding diathesis in a patient with FXIII deficiency who developed inhibitors, and to follow the clinical course of the disease with FXIII concentrate and immunosuppression.
凝血因子 XIII（FXIII）是一种纤维蛋白稳定因子，在伤口愈合和蜕膜与胎儿之间的相互作用中具有额外的作用。先天性 FXIII 缺乏症是一种罕见的出血性疾病。遗传性 FXIII 缺乏症中针对 FXIII 的抑制剂的发展也不常见，但可能导致严重的、危及生命的出血。FXIII 是凝血级联反应的最后一步，凝血参数（PT、aPTT）正常，因此 FXIII 抑制剂的检测相当困难。由于缺乏旁路制剂（如 FVII）的作用，先天性 FXIII 缺乏症伴抑制剂患者的治疗具有挑战性。在这些情况下，最著名的治疗方法是使用高剂量 FXIII 浓缩物和免疫抑制。在此，我们报告了一名 FXIII 缺乏症伴抑制剂的患者术后出血性素质的管理，并使用 FXIII 浓缩物和免疫抑制来监测疾病的临床过程。
