Ylmaz Sebnem, Oren Hale, Irken Gülersu, Atabay Berna, Duman Murat, Ylmaz Ebru, Cakmakç Handan
Department of Pediatric Hematology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey.
J Pediatr Hematol Oncol. 2005 Jul;27(7):400-2. doi: 10.1097/01.mph.0000174241.03038.b5.
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.
作者描述了一名患有甲型血友病且抑制剂滴度高的11岁男孩，他发生了危及生命的纵隔-腹膜后出血。胸部CT显示一个大血肿始于气管后区域，充满纵隔，压迫气管隆突，并腹膜后延伸至肾脏。由于手术方法死亡率高，作者最初选择了更保守的方法，使用重组活化因子VII实现了出色的出血控制，无需手术干预。正如其他患者报道的那样，作者还表明，在该患者成功接受旁路制剂治疗时，因子VIII抑制剂有所下降。
