Successful immunosuppressive therapy in female hemophilia A developing inhibitor after perioperative administration of factor VIII products
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- YAMAGUCHI Maki
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- TAKAKI Yusuke
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- YAMASAKI Yoshitaka
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- OYA Shuki
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- NAKAMURA Takayuki
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- MORISHIGE Satoshi
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- AOYAMA Kazutoshi
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- MOURI Fumihiko
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- TAKASE Ryuta
- Department of Pediatrics, Kurume University
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- MATSUO Yoko
- Department of Pediatrics, Kurume University
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- OSAKI Koichi
- Department of Transfusion, St. Mary’s Hospital
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- NAGAFUJI Koji
- Division of Hematology and Oncology, Department of Medicine, Kurume University
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- OKAMURA Takashi
- Hematology and Oncology Center, St. Mary’s Hospital
Bibliographic Information
- Other Title
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- 手術時第VIII因子製剤投与後に生じたインヒビターに対し免疫抑制療法が奏効した女性血友病A
Abstract
<p>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.</p>
Journal
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- Rinsho Ketsueki
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Rinsho Ketsueki 65 (2), 90-94, 2024
The Japanese Society of Hematology