Successful treatment of acquired factor VIII deficiency in a child using activated factor VII concentrates: case report and review of the literature.
Acquired factor VIII deficiency is a rare life-threatening disorder that should be suspected in individuals without a prior bleeding history who present with mucous membrane, muscle, and/or urinary tract bleeding. The authors describe a 5-year-old girl with epistaxis, intramuscular bleeding, and forearm compartment syndrome requiring emergent fasciotomy. Coagulation studies showed a factor VIII level of less than 1%. The prolonged activated partial thromboplastin time corrected immediately when mixed with normal plasma at a 1:1 ratio but became prolonged again following incubation at 37 degrees C. She was treated successfully with serial administrations of activated factor VII concentrates and immunosuppression with corticosteroids. Activated factor VII concentrates should be considered as an option for patients of all ages with acquired factor VIII deficiency.
Bruggers
Carol S
CS
Pediatric Hematology-Oncology, Primary Children's Medical Center, 100 North Medical Drive, Salt Lake City, UT 84113, U.S.A. pccbrugg@ihc.com
Bleak
Shirley
S
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D015942:Factor VIIa