[用凝血因子VIII替代半珠单抗治疗抑制剂活性降低的先天性血友病a患者创伤性肌内出血的成功治疗]。

Takeshi Kageyama, Makiko Mizuguchi, Yasunobu Okamoto, Hikaru Yagi, Kumiko Kagawa, Hironobu Shibata, Shuji Ozaki
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引用次数: 0

摘要

患者是一名45岁的男性,儿童期被诊断为严重血友病a,并接受了FVIII替代治疗,但由于抑制剂产生(5-225 BU/ml)而无效。在开始emicizumab治疗后,出血症状明显改善,但由于跌倒,他在右大腿出现肌肉内血肿。他住院并卧床休息;然而,血肿的大小增加,并出现贫血。由于抑制剂水平在0.6 BU/ml时明显降低,因此给予重组FVIII制剂,血肿的大小随着FVIII活性的增加而减小。抑制剂水平升高至54.2 BU/ml,但在持续治疗期间趋于下降。Emicizumab治疗似乎对产生抑制剂的A型血友病患者有用。
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[Successful treatment of traumatic intramuscular hemorrhage with coagulation factor VIII replacement in a patient with congenital hemophilia A with decreased inhibitor activity by emicizumab therapy].

The patient is a 45-year-old man who was diagnosed with severe hemophilia A during childhood and received FVIII replacement therapy, which became ineffective due to inhibitor production (5-225 BU/ml). After initiating emicizumab therapy, bleeding symptoms markedly improved, but he developed an intramuscular hematoma at the right thigh due to a fall. He was hospitalized and maintained on bed rest; however, the size of the hematoma increased, and anemia developed. Since the inhibitor level was markedly decreased at 0.6 BU/ml, a recombinant FVIII preparation was administered, and the size of the hematoma decreased along with an increase in FVIII activity. Levels of the inhibitor increased to 54.2 BU/ml, but tended to decrease during continued emicizumab treatment. Emicizumab therapy seems useful in hemophilia A patients with inhibitor production.

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