Clinical and Imaging Characteristics of Hepatic AL Amyloidosis

Overview

This study analyzes clinical and imaging findings in 10 patients with hepatic AL amyloidosis. Key manifestations include hepatomegaly, elevated liver enzymes, and specific imaging characteristics.

Background

Hepatic AL amyloidosis is a rare but serious condition characterized by the deposition of amyloid proteins in the liver, leading to organ dysfunction. Early recognition is critical as it often presents with nonspecific symptoms, resulting in diagnostic delays.

Data Highlights

Key Findings

• All patients presented with hepatomegaly (100%).
• Laboratory findings included markedly elevated alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) in all patients.
• CT imaging showed diffuse hepatomegaly with decreased hepatic attenuation in 8 patients.
• MRI revealed marked hepatomegaly with smooth margins and vascular rarefaction in 6 patients.
• Delayed enhancement was noted on contrast-enhanced imaging.
• Splenic hypoperfusion was observed in 7 patients.

Clinical Implications

Healthcare professionals should maintain a high index of suspicion for hepatic AL amyloidosis in patients presenting with unexplained hepatomegaly and elevated liver enzymes.

Conclusion

Hepatic AL amyloidosis requires prompt recognition based on clinical and imaging clues.

Related Resources & Content

1. Blood Cancer Journal, 2021 -- Guidelines for the Diagnosis and Management of Immunoglobulin Light Chain Amyloidosis
2. European Radiology, 2024 -- Hepatobiliary Phase MRI with Gadoxetic Acid Reveals Lesions with Hyper- to Isointense Characteristics Compared to Adjacent Liver Tissue
3. European Radiology, 2025 -- Imaging Characteristics of Newly Recognized Low-Grade Vascular Tumors in the Liver: Hepatic Small Vessel Neoplasm and Anastomosing Hemangioma
4. Blood Cancer Journal, 2022 -- Results of Kidney Transplantation in Patients with AL Amyloidosis
5. ASH CPG Amyloidosis Visual Summary 0626 -- Clinical Guidelines
6. ASH CPG Amyloidosis Visual Summary 0626
7. Daratumumab-Bortezomib-Cyclophosphamide-Dexamethasone in Newly Diagnosed Amyloidosis: ANDROMEDA Final Survival Analysis - ScienceDirect
8. Imaging for abdominal involvement in amyloidosis - PMC