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Prophylactic recombinant factor VIIa administration to an infant with congenital systemic juvenile xanthogranuloma

Jesus De Santiago1, Ernesto Martinez-Garcia, Jorge Giron

  • 1Department of Anesthesiology, Children University Hospital Niño Jesus, Madrid, Spain. jdesantiago@telefonica.net

Paediatric Anaesthesia
|August 22, 2006
PubMed

Insights

Recombinant factor VIIa (rFVIIa) effectively prevented bleeding during invasive procedures in an infant with congenital systemic juvenile xanthogranuloma. This therapy proved successful when other treatments for coagulopathy failed.

Area of Science:

  • Hematology
  • Pediatrics
  • Oncology

Background:

  • Congenital systemic juvenile xanthogranuloma (s-XG) can present with significant coagulopathy.
  • Infants with s-XG may require invasive procedures like central venous access system implantation and biopsies.
  • Managing coagulopathy refractory to standard treatments poses a clinical challenge.

Observation:

  • A pediatric patient with s-XG exhibited impaired liver function, thrombocytopenia, and refractory coagulopathy.
  • The patient was scheduled for central venous access system implantation and liver/bone marrow biopsies.
  • Standard treatments including fresh-frozen plasma and platelet infusions were ineffective.

Findings:

  • Recombinant factor VIIa (rFVIIa) was administered prophylactically at 80 microg/kg every 2 hours.
  • rFVIIa was given pre-procedure, intra-procedure, and post-procedure (30 min before to 6 hours after).
  • Only minor bleeding was observed during the invasive procedures.

Implications:

  • rFVIIa can be an effective prophylactic agent for invasive procedures in pediatric patients with refractory coagulopathy.
  • This case highlights a potential therapeutic option for managing bleeding risks in complex pediatric cases.
  • Successful rFVIIa use may improve outcomes for children with s-XG undergoing necessary interventions.

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