Department of Orthopaedic Surgery, Hokkaido University Graduate School of Medicine, North 15 West 7, Sapporo, 060-8638, Japan.
Int J Hematol. 2012 Dec;96(6):781-5. doi: 10.1007/s12185-012-1191-x. Epub 2012 Oct 16.
Factor XIII (FXIII) is the final enzyme in the coagulation cascade. Acquired FXIII deficiency is caused by inhibitors of FXIII or decreased synthesis and/or increased consumption of FXIII, which leads to severe bleeding. Recently, we experienced a case of hemorrhagic-acquired factor XIII deficiency that occurred during treatment with the IL-6 inhibitor tocilizumab for rheumatoid arthritis. A 48-year-old man was referred because of right hip pain due to a hematoma. Laboratory findings showed that routine coagulation tests were normal, while FXIII activity was slightly low (52.4 %). The patient was successfully treated with plasma-derived factor XIII concentrates. The time course of recovery suggests that tocilizumab might have inhibited FXIII production. To our knowledge, this is the first report of acquired factor XIII deficiency associated with administering of tocilizumab. When recurrent bleeding is seen during administering of tocilizumab, acquired factor XIII deficiency may have been induced, thus attending physicians should consider this disease in a differential diagnosis.
凝血级联反应的最后一个酶是因子 XIII（FXIII）。获得性 FXIII 缺乏症是由 FXIII 抑制剂或 FXIII 合成减少和/或消耗增加引起的，这会导致严重出血。最近，我们遇到了一例在使用 IL-6 抑制剂托珠单抗治疗类风湿关节炎时发生的获得性因子 XIII 缺乏症出血病例。一名 48 岁男性因右髋血肿疼痛而被转介。实验室检查结果显示常规凝血试验正常，而 FXIII 活性略低（52.4%）。该患者成功接受了血浆源性因子 XIII 浓缩物治疗。恢复的时间过程表明，托珠单抗可能抑制了 FXIII 的产生。据我们所知，这是首例与托珠单抗给药相关的获得性因子 XIII 缺乏症的报告。当在托珠单抗给药期间出现反复出血时，可能已经诱导了获得性因子 XIII 缺乏症，因此主治医生应在鉴别诊断中考虑该病。
