[女性 A 型血友病患者在围手术期使用 VIII 因子产品后出现抑制因子,成功接受免疫抑制治疗]。

Maki Yamaguchi, Yusuke Takaki, Yoshitaka Yamasaki, Shuki Oya, Takayuki Nakamura, Satoshi Morishige, Kazutoshi Aoyama, Fumihiko Mouri, Ryuta Takase, Yoko Matsuo, Koichi Osaki, Koji Nagafuji, Takashi Okamura
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引用次数: 0

摘要

一名 62 岁的妇女在卵巢肿瘤术前检查中被诊断为 A 型血友病携带者(VIII因子活性为 35%)。围手术期总共使用了 35,600 单位的重组因子 VIII 产品。术后第 95 天,皮下血肿形成,根据 APTT 为 66 秒、因子 VIII (FVIII) 活性为 3% 和 FVIII 抑制剂为 1 BU/ml,开始使用泼尼松龙进行免疫抑制治疗。治疗期间,患者因踝关节出血住院,需要止血治疗,但术后第 438 天服用环磷酰胺后,抑制剂消失,FVIII 活性恢复到 30%。F8 分析显示该患者携带 p.Arg391Cys 杂合子,该杂合子以前被归类为交叉反应物质(CRM)阳性重症 A 型血友病。我们还报告了对该患者进行醋酸去氨加压素水合物试验的结果,该试验的目的是为将来发生大出血时的治疗做准备,因为大剂量 FVIII 给药可能是抑制剂产生的一个因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Successful immunosuppressive therapy in female hemophilia A developing inhibitor after perioperative administration of factor VIII products].

A 62-year-old woman was diagnosed as a hemophilia A carrier (factor VIII activity 35%) on preoperative examination of an ovarian tumor. A total of 35,600 units of recombinant factor VIII products was administered perioperatively. On postoperative day 95, a subcutaneous hematoma formed and immunosuppressive therapy with prednisolone was started based on an APTT of 66 seconds, factor VIII (FVIII) activity of 3%, and FVIII inhibitor of 1 BU/ml. During this treatment, the patient was hospitalized due to ankle joint bleeds and required hemostatic treatment, but the inhibitor disappeared and FVIII activity recovered to 30% after postoperative day 438 with cyclophosphamide. F8 analysis revealed the patient carried a heterozygosity of p.Arg391Cys, which has previously been categorized as cross-reacting material (CRM)-positive severe hemophilia A. No high-risk mutations for inhibitor development were found. We also report the results of a desmopressin acetate hydrate test administered to the patient to prepare for future treatment in case of hemorrhage, since high-dose FVIII administration may have been a factor in inhibitor development.

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