Objective: Coagulopathy resulting from liver pathologies and the hemorrhages secondary to this are the complications observed in chronic liver diseases, fulminant hepatitis, hepatocellular cancer, liver transplantation and other similar conditions. The literature on the use of rFVIIa which was initially used in hemophiliac patients with inhibitors for hemorrhages that can not be managed with conventional methods or operations that can not be performed safely, is increasingly growing. Here we will present a patient that was successfully operated by using rFVIIa for recurrent intracranial hemorrhage secondary to liver disease.
Case: The 7 months old female infant was brought to our clinic due to abdominal distention and bleeding from anus. Her medical history included a treatment by hospitalization in the Regional Hospital when two months old due to high fever, forceful vomiting, foaming at mouth and yellow coloring of skin (convulsion?), operation due to hemorrhage detected at cerebral imaging, two subsequent operations at the same hospital due to hemorrhage upon sudden purple coloring of skin observed 1.5 months later and finally, dismission from hospital following a 25-day monitoring under conditions of intensive care. Following the yellow coloring of eyes, hematemesis, abdominal distention, echhymoses in the back and chest observed in the patient, she was referred to our clinic and her cerebral CT findings revealed pressure effect and subdural hematoma extending from temporal localization to frontoparietal cranium at left and to tentorium superior section and anterior interhemispheric fissure in cranium at posterior, with the widest part reaching 1.5 cm. The patient, for whom an operation was planned, was twice supplemented with platelet suspension at a dose of 10 cc/kg (53000/mm3) for her thrombocytopenia. Despite the administration of Frozen Plasma supplement, PT, PTT and Fibrinogen was detected as 18, 42.8 and 117 mg/dl, respectively and 1.2 mg of Novoseven was administered to the patient once preoperatively and twice postoperatively at a two-hour interval due to her history of postoperative hemorrhage. Upon the preoperative detection of PT, PTT, Fibrinogen as 12.1, 43.1 and 145 mg/dl and KZ and PZ as 6 and 5 minutes respectively, the patient was operated. No new postoperative hemorrhages or other complications were observed. The diagnosis of Gaucher disease was made at the bone marrow examination and the relevant treatment was planned.
Conclusion: rFVIIa can be safely used in high-risk patients with a history of recurrent hemorrhage, for whom no improvement can be achieved in the hemostasis tests.