Life-threatening mediastinal-retroperitoneal hemorrhage in a child with moderate hemophilia A and high inhibitor titer: successful management with recombinant activated factor VII.
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.
Ylmaz
Sebnem
S
Department of Pediatric Hematology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey.
Oren
Hale
H
Irken
Gülersu
G
Atabay
Berna
B
Duman
Murat
M
Ylmaz
Ebru
E
Cakmakç
Handan
H
D000328:Adult; D005167:Factor VII / Q000627:therapeutic use*; D006406:Hematoma / Q000000981:diagnostic imaging; D006465:Hemoperitoneum / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D008297:Male; D013902:Radiography, Thoracic; D011994:Recombinant Proteins / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D011994:Recombinant Proteins; D005167:Factor VII