Plasma-derived Factor X therapy for treatment of intracranial bleeding in a patient with Factor X deficiency: a case report.

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  • Additional Information
    • Source:
      Publisher: American Association Of Blood Banks Country of Publication: United States NLM ID: 0417360 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1537-2995 (Electronic) Linking ISSN: 00411132 NLM ISO Abbreviation: Transfusion Subsets: MEDLINE
    • Publication Information:
      Original Publication: Arlington, Va. : American Association Of Blood Banks
    • Subject Terms:
      Factor X/*therapeutic use ; Factor X Deficiency/*complications ; Intracranial Hemorrhages/*etiology; Consanguinity ; Factor X/administration & dosage ; Factor X Deficiency/genetics ; Female ; Humans ; Infant ; Intracranial Hemorrhages/diagnosis ; Intracranial Hemorrhages/diagnostic imaging ; Magnetic Resonance Imaging ; Mutation, Missense
    • Abstract:
      Background: Factor X (FX) deficiency (FXD) is an extremely rare autosomal recessive hereditary hematologic disorder, affecting approximately one in 1,000,000 of the general population.
      Case Report: This case report describes an infant with hereditary severe FXD who presented with a spontaneous, life-threatening intracranial hemorrhage and was treated with the first licensed plasma-derived FX (pdFX) concentrate. On admission, laboratory assays showed severe coagulopathy of unknown cause; the patient was empirically treated using a multimodal hemostatic approach with prothrombin complex concentrate, fresh-frozen plasma, and tranexamic acid. Subsequent single-factor coagulation and genetic analyses confirmed the hereditary FXD diagnosis, and the therapeutic regimen was changed to a targeted regimen of 250 IU pdFX daily. Based on careful monitoring of the coagulation profile, pdFX administration frequency was increased to twice daily, followed by a reduction to once every 18 hours. The patient was discharged after 7 weeks of hospitalization in good clinical condition and now receives prophylactic pdFX three times weekly.
      (© 2019 AABB.)
    • Accession Number:
      9001-29-0 (Factor X)
    • Publication Date:
      Date Created: 20190410 Date Completed: 20200602 Latest Revision: 20200602
    • Publication Date:
      20231215
    • Accession Number:
      10.1111/trf.15308
    • Accession Number:
      30964547