Intra-hepatic haemorrhage and shock during post-natal period, in two brothers with haemophilia

Abstract

A boy, born at term by vaginal delivery, developed coagulopathy and hypovolaemic shock at 15 hours. pH was 6.66, lactate 21 mmol/l, haemoglobin 8.9 g/dl, platelets 178x10 9 /l, APTT 245 seconds, PT 21 seconds, fibrinogen 0.9 g/l. He was ventilated and received O-negative blood, plasma, vitamin K, antibiotics and inotropic support. He responded to treatment and was extubated after eight hours. Coagulation normalised but he required further blood and platelet transfusions. He developed abdominal distention with hepatomegaly. Ultrasound images suggested a hepatic tumour with haemorrhage into the tumour and the peritoneal cavity (Figure 1). He required further blood and platelets transfusions. By day thirteen, the ultrasound appearances were consistent with a resolving hematoma. Repeated coagulation screens showed prolonged APTT. Factor VIII level of <0.01 IU/ml confirmed a diagnosis of haemophilia A.