'Fragile' liver and massive hepatic haemorrhage due to hereditary amyloidosis

The first case of amyloidosis is reported in which spontaneous massive hepatic haemorrhage necessitated emergency Liver transplantation. Liver transplantation, as a treatment for a failing liver due to amyloidosis has not been previously reported. At transplantation, the liver tissue was uncharacteristically friable, although the subsequent vascular and biliary anastomoses were uncomplicated. Histological examination of the Liver showed a surprisingly modest amount of amyloid, which was shown immunohistochemically to be derived from lysozyme, and a striking absence of reticulin staining. Both the patient's father and paternal grandfather had died from spontaneous hepatic haemorrhage, and histological review of their liver tissue showed similarly modest deposition of lysozyme-derived amyloid associated with loss of reticulin staining. In each case the quantity of amyloid was far less than would be expected to interfere with the mechanical integrity of the liver. This is the only report of hepatic disintegration associated with absence of reticulin staining, and it is probable that the mechanism represents a novel secondary effect of the amyloid deposits in the livers of this family.