一例体弱老年患者自身免疫因子XIII缺乏的治疗

IF 1.2 4区 医学 Q4 HEMATOLOGY
Masahiro Kaneko, Naoto Ishimaru, Takahiro Nakajima, Yohei Kanzawa, Hiroyuki Seto, Saori Kinami, Tsukasa Osaki, Masayoshi Souri, Akitada Ichinose
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引用次数: 0

摘要

自身免疫因子XIII/13缺乏症(aFXIII缺乏症)是一种罕见的出血性疾病,典型的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,对这类患者的最佳管理尚未达成共识。我们的老年患者有大量肌肉内血肿,诊断为aFXIII缺乏。患者选择了积极的免疫抑制治疗,因此他只接受保守治疗。在类似病例中,还需要对其他可纠正的出血和贫血原因进行彻底调查。我们的病人的血清素-去甲肾上腺素再摄取抑制剂的使用和多种维生素缺乏(维生素C, b12和叶酸)被发现是加重因素。预防跌倒和预防肌肉压力对老年患者也很重要。患者6个月内两次出血复发,经卧床休息自行好转,未使用XIII因子替代治疗或输血。保守管理可能更适合体弱和老年患者与aFXIII缺乏症时,他们选择反对标准治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Management of autoimmune factor XIII deficiency in a frail, elderly patient.

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.

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来源期刊
CiteScore
1.90
自引率
0.00%
发文量
111
审稿时长
4-8 weeks
期刊介绍: Blood Coagulation & Fibrinolysis is an international fully refereed journal that features review and original research articles on all clinical, laboratory and experimental aspects of haemostasis and thrombosis. The journal is devoted to publishing significant developments worldwide in the field of blood coagulation, fibrinolysis, thrombosis, platelets and the kininogen-kinin system, as well as dealing with those aspects of blood rheology relevant to haemostasis and the effects of drugs on haemostatic components
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