Kageyama Takeshi, Mizuguchi Makiko, Okamoto Yasunobu, Yagi Hikaru, Kagawa Kumiko, Shibata Hironobu, Ozaki Shuji
Center for clinical education, Tokushima Prefectural Central Hospital.
Department of Hematology, Tokushima Prefectural Central Hospital.
Rinsho Ketsueki. 2023;64(3):198-202. doi: 10.11406/rinketsu.64.198.
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.
该患者为一名45岁男性,童年时期被诊断为重度甲型血友病,并接受了FVIII替代疗法,但由于产生抑制剂(5 - 225 BU/ml)而变得无效。开始使用依美珠单抗治疗后,出血症状明显改善,但他因跌倒在右大腿出现了肌内血肿。他住院并卧床休息;然而,血肿大小增加,且出现了贫血。由于抑制剂水平显著下降至0.6 BU/ml,给予了重组FVIII制剂,血肿大小随着FVIII活性的增加而减小。抑制剂水平升至54.2 BU/ml,但在继续使用依美珠单抗治疗期间趋于下降。依美珠单抗治疗对于产生抑制剂的甲型血友病患者似乎是有用的。