Amano Shiho, Oka Kohei, Sato Yutaka, Sano Chiaki, Ohta Ryuichi
Community Care, Unnan City Hospital, Unnan 699-1221, Japan.
Department of Community Medicine Management, Faculty of Medicine, Shimane University, Izumo 693-8501, Japan.
J Clin Med. 2022 Mar 18;11(6):1699. doi: 10.3390/jcm11061699.
Factor XIII (FXIII) deficiency is a rare but serious coagulopathy. FXIII is critical in blood coagulation, and FXIII deficiencies can lead to uncontrolled or spontaneous bleeding. FXIII deficiencies can be congenital or acquired; acquired FXIII deficiency can be categorized as autoimmune and non-autoimmune. Immunological tests to measure FXIII inhibitors are required to diagnose acquired FXIII deficiency; however, appropriate test facilities are limited, which increases the turnaround time of these tests. In the case of critical bleeding, delayed test results may worsen prognosis due to delayed treatment. Here, we report a case of acquired FXIII deficiency, followed by a review of FXIII deficiency cases in Japan. We performed a systematic review to investigate the present conditions of the diagnosis and treatment of FXIII deficiency, including the measurement of FXIII inhibitors in Japan. FXIII inhibitor testing was only performed in 29.7 of acquired FXIII deficiency cases. Clinical departments other than internal medicine and pediatrics were often involved in medical treatment at the time of onset. Therefore, it is important for doctors in clinical departments other than internal medicine and pediatrics to consider FXIII deficiency and perform FXIII inhibitor testing when examining patients with prolonged bleeding of unknown cause or persistent bleeding after trauma.
因子 XIII（FXIII）缺乏症是一种罕见但严重的凝血病。FXIII 在血液凝固中起关键作用，FXIII 缺乏可导致不受控制的出血或自发性出血。FXIII 缺乏症可分为先天性或后天性；后天性 FXIII 缺乏症可分为自身免疫性和非自身免疫性。诊断后天性 FXIII 缺乏症需要进行测量 FXIII 抑制剂的免疫学检测；然而，合适的检测设施有限，这增加了这些检测的周转时间。在严重出血的情况下，检测结果延迟可能会因治疗延迟而使预后恶化。在此，我们报告一例后天性 FXIII 缺乏症病例，并对日本的 FXIII 缺乏症病例进行综述。我们进行了一项系统评价，以调查日本 FXIII 缺乏症的诊断和治疗现状，包括 FXIII 抑制剂的检测。在后天性 FXIII 缺乏症病例中，仅 29.7%进行了 FXIII 抑制剂检测。发病时，内科和儿科以外的临床科室常参与医疗救治。因此，对于内科和儿科以外临床科室的医生来说，在检查原因不明的长期出血患者或创伤后持续出血患者时，考虑 FXIII 缺乏症并进行 FXIII 抑制剂检测非常重要。
