Response of Bortezomib Chemotherapy in Hepatic Amyloidosis

摘要

Amyloidosis is a rare disorder with a wide spectrum of presentations and anomalies. It is subdivided into 2 broad categories based on protein deposition; primary and secondary amyloidosis. It can present as a single-organ involvement or as a diffuse infiltrative multi-organ process. Isolated hepatic amyloidosis presentation is a rare phenomenon that develops due to insoluble amyloid deposition in liver. Its clinical presentation is usually vague and ranges from mild hepatomegaly with elevated liver enzymes to acute liver failure and hepatic rupture. Currently, there are scarce data available regarding treatment options for biopsy-proven hepatic amyloidosis. In this review article, we present an interesting case of hepatic amyloidosis and its poor outcome to new molecular targeted chemotherapy. Furthermore, we aim to review current and future diagnostic tools for early detection and advancements in targeted chemotherapeutics options available for hepatic amyloidosis.