[Successful immunosuppressive therapy in female hemophilia A developing inhibitor after perioperative administration of factor VIII products].
Rinsho Ketsueki
; 65(2): 90-94, 2024.
Article
in Ja
| MEDLINE
| ID: mdl-38448004
ABSTRACT
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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Hemostatics
/
Hemophilia A
Limits:
Female
/
Humans
/
Middle aged
Language:
Ja
Journal:
Rinsho Ketsueki
Year:
2024
Document type:
Article
Country of publication:
Japan