Successful treatment of hemothorax and intracranial hemorrhage in a taiwanese hemophiliac with inhibitors by activated recombinant factor vii and positive pressure ventilation

Introduction: Hemothorax is potentially lethal with increasing mortality rate of four-fold when combined with intracranial hemorrhage (ICH). It is rare in hemophiliacs. Pleural movement in respiration causes it difficult to stop hemorrhage into pleural space. When hemothorax occurs in a hemophiliac with inhibitors, it is more risky and extremely rare. There have been only three reported cases. We report such a case with traumatic hemothorax with ICH, successfully treated with recombinant factor VIIa (rFVIIa) and positive pressure ventilation (PPV) which has been not noted in previous three cases, and review literature. Case presentation: The 48 y/o male with severe hemophilia A and high-titer inhibitors was admitted with drowsiness in 2010, because he fell down from stairs to ground. ICH and rightside hemothorax were found. rFVIIa 90 mcg/kg/dose q2h was administrated, with intensive care. Due to hemoglobin (Hb) dropped from 14.5 to 6.8 g/dL and platelet count dropped from 140000/ul to 40000/ul, and due to hypofibrinogenemia, PRBC, platelet, and cryoprecipitate were repeatedly transfused. Due to respiratory failure, endotracheal tube (ETT) was intubated. However, under pressurecontrolled ventilation, Hb still dropped despite use of rFVIIa. Repeat CT scan showed stabilized ICH but exacerbated hemothorax. When high PEEP pressure-control with increased sensitivity of flow trigger was utilized, Hb level started to be elevated. Finally, ETT was weaned. rFVIIa was tapered, with total 143 doses in this 21-day hospitalization. Neither recurrence nor sequelae occurred during the follow- up period of 6 months. Conclusions: rFVIIa is effective for hemothorax with ICH in the hemophiliac with inhibitors. PPV can increase positive end-expiratory pressure for tamponade of pleural space, causing successful hemostasis and weaning from ventilator. Monitoring clinical conditions and laboratory data, e.g.: variation in Hb, can determine whether hemostasis is achieved and rFVIIa should be adjusted.