Abstract
Congenital factor VII (FVII) deficiency is a rare, autosomal recessive bleeding disorder with a spectrum of phenotypes ranging from asymptomatic to life-threatening intra-cranial hemorrhage (ICH). Orthotopic liver transplantation has been described for definitive treatment in a few patients with severe manifestations. We report a patient with congenital FVII deficiency and recurrent ICH, despite twice-weekly prophylaxis with recombinant activated FVII. At 17 months of age, he underwent an orthotopic liver transplant. He is now 1-year post-transplant, on maintenance immunosuppression with no hemorrhage or other complications.
Original language | English (US) |
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Pages (from-to) | 1886-1887 |
Number of pages | 2 |
Journal | Pediatric Blood and Cancer |
Volume | 61 |
Issue number | 10 |
DOIs | |
State | Published - Oct 2014 |
Keywords
- Factor VII deficiency
- Liver transplant
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Hematology
- Oncology