Life-threatening mediastinal-retroperitoneal hemorrhage in a child with moderate hemophilia A and high inhibitor titer - Successful management with recombinant activated factor VII

Yilmaz B., Oren H., Irken G., Atabay B., DUMAN M., Yilmaz E., ...More

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, vol.27, no.7, pp.400-402, 2005 (SCI-Expanded) identifier

  • Publication Type: Article / Article
  • Volume: 27 Issue: 7
  • Publication Date: 2005
  • Journal Name: JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.400-402
  • Keywords: hemophilia A, factor VIII inhibitor, mediastinal and retroperitoneal bleeding, recombinant activated factor VII, FACTOR-IX INHIBITORS, SPONTANEOUS HEMOTHORAX, GENOTYPE, MILD, HEMOMEDIASTINUM, PHENOTYPE, RFVIIA
  • Dokuz Eylül University Affiliated: Yes

Abstract

The authors describe an 11-year-old boy with hemophilia A and high titer inhibitor who developed a life-threatening mediastinal-retroperitoneal hemorrhage. Chest CT showed a large hematoma beginning in the retrotracheal area, filling the mediastinum, compressing the carina, and extending retroperitoneally up to the kidneys. As the surgical approach has a high mortality rate, the authors chose a more conservative approach initially and obtained excellent bleeding control with recombinant activated factor VII without the need for surgical intervention. As reported in other patients, the authors also showed a decrease in the factor III inhibitor while this patient was successfully treated with bypassing agents.