(PA-334) Successful Liver Transplantation for Acute Liver Failure Caused by AL Amyloidosis

Systemic light chain (AL) amyloidosis is a rare, life-threatening condition marked by insoluble amyloid fibril deposition in tissues, leading to organ dysfunction. While the heart and kidneys are most frequently affected, severe hepatic involvement remains rare and is linked to poor outcomes. Often associated with multiple myeloma, AL amyloidosis poses significant therapeutic challenges. We report a rare case of fulminant hepatic failure caused by AL amyloidosis associated with indolent multiple myeloma, successfully managed with liver transplantation and systemic therapy.

Methods:   

In February 2024, a 58-year-old man with no significant medical history presented with progressive fatigue and weight loss. In the following months, blood tests revealed isolated thrombocytosis, followed by cholestasis and hepatic cytolysis. A transjugular liver biopsy showed Congo red-positive deposits, confirming hepatic amyloidosis.

Upon admission to our centre in mid-July 2024, the patient presented with acute liver failure, characterised by hyperbilirubinemia, severe cytolysis, and hemorrhagic ascites. Serum protein electrophoresis revealed an IgG lambda monoclonal peak of 8.1 g/L and a reduced free light chain kappa/lambda ratio of 0.06. A bone marrow biopsy indicated indolent multiple myeloma with amyloid deposits. Further investigations showed no cardiac or renal amyloid involvement.

Treatment with daratumumab and dexamethasone, according to the D-VCD protocol, was initiated promptly. However, the patient's condition deteriorated so rapidly that an emergency liver transplant was considered. Performed on 27 July 2024, the procedure enabled a swift recovery of liver function. One month later, serum lambda light chains had dropped from 242 mg/L to 85 mg/L. Bortezomib was added at reduced dose from the second treatment cycle, leading to VGPR. The patient was discharged in mid-August 2024. Liver biopsies at six months showed no evidence of amyloid infiltration. He is now in the maintenance phase of treatment and has resumed normal professional activity.

Results:   

This case illustrates a rare instance of AL amyloidosis with severe liver involvement successfully managed by liver transplantation and systemic therapy. Acute liver failure necessitated urgent multidisciplinary care. The transplant restored hepatic function, and systemic therapy rapidly improved haematological response and reduced amyloid burden.

Unlike transthyretin (ATTR) amyloidosis, where liver transplantation is well established, its role in AL amyloidosis remains exceptional. This is due to the rarity of isolated hepatic involvement and frequent cardiac comorbidities, which often preclude transplantation. AL amyloidosis usually presents with significant systemic burden, making patient selection and perioperative management challenging.

 

Conclusions:   

This case highlights the potential of combining liver transplantation with early systemic therapy as a life-saving approach for certain patients with AL amyloidosis and acute liver failure.