Kadono Moe, Souri Masayoshi, Shimomura Takeshi, Kuroda Yoshiaki, Munemasa Shoso, Ozaki Tsukasa, Ichinose Akitada
Department of Hematology, National Hospital Organization Hiroshimanishi Medical Center.
Department of Molecular Pathobiochemistry and Pathobiology, Yamagata University School of Medicine.
Rinsho Ketsueki. 2023;64(12):1508-1513. doi: 10.11406/rinketsu.64.1508.
An 88-year-old man became unconscious and was admitted to our hospital due to severe anemia. Extensive subcutaneous hemorrhage around the chest and back and pectoralis major muscle hematoma were observed. Coagulation screening tests showed moderately reduced factor XIII/13 (FXIII) activity. During hospitalization, the patient had repeated bleeding events in the gastrointestinal tract and muscles, leading to hemorrhagic shock. We suspected the presence of FXIII inhibitors from FXIII infusion test results. The cross-mixing test for cross-linking of fibrin revealed inhibition of polymerization of α-chain and α-plasmin inhibitor incorporation into fibrin. In addition, by detecting IgG autoantibody to thrombin-activated FXIII, we confirmed the presence of type Ab anti-FXIII-A subunit autoantibody, which represses the catalytic subunit activity of activated FXIII. Autoimmune FXIII deficiency should be considered when a patient presents with severe hemorrhagic diathesis with no other cause than moderately reduced of FXIII activity, as reported in this case.
一名88岁男性因严重贫血而昏迷，被收治入我院。观察到胸部、背部广泛皮下出血及胸大肌血肿。凝血筛查试验显示因子XIII/13（FXIII）活性中度降低。住院期间，患者胃肠道和肌肉反复发生出血事件，导致失血性休克。根据FXIII输注试验结果，我们怀疑存在FXIII抑制剂。纤维蛋白交联的交叉混合试验显示α链聚合及α-纤溶酶抑制剂掺入纤维蛋白受到抑制。此外，通过检测针对凝血酶激活的FXIII的IgG自身抗体，我们证实存在Ab型抗FXIII-A亚基自身抗体，该抗体可抑制活化FXIII的催化亚基活性。如本病例报告所述，当患者出现严重出血素质且无其他原因仅FXIII活性中度降低时，应考虑自身免疫性FXIII缺乏症。
