Introduction: Amyloidosis has two principle types: multiple myeloma-associated (AL) and amyloidassociated amyloidosis (AA). Both types can be local or systemic and can often involve the liver. Hepatic amyloidosis carries a poor prognosis, but commonly presents with a more gradual time course of evolving hepatomegaly and elevated serum alkaline phosphatase. This casereport involves an unusual presentation of hepatic amyloidosis with acute liver decompensation resulting in death.
Case presentation: A 76 year old man presented with complaints of progressive weakness and anorexia resulting in weight loss. He was initially found to have laboratory abnormalities notable for cholestasis and a mild transaminitis. On exam, the patient was cachectic with scleral icterus, jaundice, and abdominal distention. Liver ultrasound with doppler imaging was significant for increased echogenicity of the liver and ascites. The patient underwent transjugular liver biopsy, which demonstrated hepatic amyloid. Additional testing revealed a diagnosis of amyloidosis secondary to multiple myeloma. On day 5 of the patient's admission, cholestasis and transaminitis acutely worsened, with eventual peaking of aspartate aminotransferase to 3362 u/l, alanine aminotransferase to 1439 u/l, and total bilirubin to 18.8 mg/d. Further work-up for this acute decompensation, including imaging and laboratory tests, did not reveal any triggering events such as portal vein thrombosis or intra-abdominal bleeding from recent liver biopsy. Additionally, cardiovascular compromise, infectious etiologies, and toxins were ruled out. Unfortunately, the patient was not a candidate for chemotherapy given his acute hepatic decompensation and subsequent encephalopathy on day 8 of admission. The patient was started on Lactulose and N-acetylcysteine, though his mental status continued to worsen and the patient died on day 10 of admission from multi-organ compromise secondary to acute liver failure.
Conclusion: Amyloidosis resulting in acute hepatic failure has previously been reported but remains quite rare. Clinicians must consider infiltrative diseases, such as multiple myeloma and amyloidosis, when patients present with an extended prodromal illness paired with unexplained, worsening hepatic dysfunction. Likewise, physicians should be aware that patients who present with hepatic amyloid secondary to multiple myeloma are at high risk for rapid clinical deterioration.
Keywords: Amyloidosis, acute liver failure, multiple myeloma, ascites, biopsy, encephalopathy