Spontaneous hepatic rupture due to primary amyloidosis

Abstract

Spontaneous hepatic rupture is an uncommon cause of haemorrhagic shock and very rarely happens due to amyloidosis. This report describes one such case in which a middle-aged man presented in extremis. He was managed initially with massive transfusion, interventional radiology embolisation and decompressive laparotomy for abdominal compartment syndrome. Subsequent coagulopathy was treated with activated factor VII due to deficient native activity. Serum protein electrophoresis and liver biopsy during his hospital course yielded a diagnosis of amyloidosis, which was treated palliatively with steroids and bortezomib. Despite supportive care, he died 10 days after presentation. This case illustrates the importance of considering an uncommon pathology when a patient presents with a condition in an uncommon way.