Department of General Internal Medicine, Akashi Medical Center, Hyogo.
Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University.
Blood Coagul Fibrinolysis. 2023 Sep 1;34(6):408-413. doi: 10.1097/MBC.0000000000001202. Epub 2023 Feb 7.
Autoimmune factor XIII/13 deficiency (aFXIII deficiency) is a rare hemorrhagic disorder, for which typical guideline-directed treatment is aggressive immunosuppressive therapy. Approximately 20% of patients are over 80 years old; however, and optimum management of such patients has not reached consensus. Our elderly patient had massive intramuscular hematoma, and aFXIII deficiency was diagnosed. The patient opted against aggressive immunosuppressive therapy, so he was managed with conservative treatment only. Thorough survey of other correctable causes of bleeding and anemia is also required in similar cases. Our patient's serotonin-norepinephrine reuptake inhibitor use and multivitamin deficiency (vitamin C, B 12 and folic acid) were revealed to be aggravating factors. Fall prevention and muscular stress prevention are also important in elderly patients. Our patient had two relapses of bleeding within 6 months, which were improved spontaneously by bed rest without factor XIII replacement therapy or blood transfusion. Conservative management may be preferred for frail and elderly patients with aFXIII deficiency when they opt against standard therapy.
自身免疫性因子 XIII/13 缺乏症（aFXIII 缺乏症）是一种罕见的出血性疾病，其典型的指南指导治疗是积极的免疫抑制治疗。大约 20%的患者年龄超过 80 岁；然而，对于此类患者的最佳管理尚未达成共识。我们的老年患者出现了巨大的肌肉血肿，诊断为 aFXIII 缺乏症。患者选择不进行积极的免疫抑制治疗，因此仅接受保守治疗。在类似情况下，还需要彻底调查其他可纠正的出血和贫血原因。我们患者的使用 5-羟色胺-去甲肾上腺素再摄取抑制剂和多种维生素缺乏（维生素 C、B12 和叶酸）被认为是加重因素。预防跌倒和肌肉紧张对于老年患者也很重要。我们的患者在 6 个月内出现了两次出血复发，通过卧床休息而无需因子 XIII 替代治疗或输血即可自发改善。当 aFXIII 缺乏症患者选择不进行标准治疗时，对于体弱和老年患者，保守治疗可能是首选。
