A Case of Acquired Factor XIII Deficiency with Systemic Lupus Erythematosus Diagnosed after Repeated Intracerebral Hemorrhages.

Eitaro Okumura, Hiroyuki Onuki, Kunitoshi Otsuka, Shigeki Sunaga, Asashi Tanaka, Hiroyuki Jimbo
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Abstract

We present a case of autoimmune-acquired factor XIII deficiency associated with systemic lupus erythematosus, which was diagnosed as a cause of repeated intracerebral hemorrhage. An intracerebral hemorrhage occurred in a 24-year-old female patient. Craniotomy was performed to remove the hematoma, but rebleeding occurred at the same site on days 2 and 11, respectively. Detailed blood tests revealed that factor XIII activity decreased. Although autoimmune-acquired factor XIII deficiency is a very rare disease, it can sometimes be fatal when intracerebral hemorrhage occurs. If there is repeated intracerebral hemorrhage, factor XIII activity should be confirmed.

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反复脑出血后诊断为系统性红斑狼疮的获得性因子13缺乏1例。
我们报告一例自身免疫获得性因子XIII缺乏与系统性红斑狼疮相关,被诊断为反复脑出血的原因。24岁女性患者发生脑出血。开颅切除血肿,但分别于第2天和第11天在同一部位再次出血。详细的血液检查显示十三因子活性下降。虽然自身免疫获得性因子XIII缺乏症是一种非常罕见的疾病,但当发生脑出血时,有时可能是致命的。如果反复出现脑出血,应确认因子XIII的活性。
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