Clinical and CT/MRI features of hepatic AL amyloidosis: preliminary experience in 10 cases.

PURPOSE: To identify the key clinical and imaging clues on computed tomography (CT) and magnetic resonance imaging (MRI) that can suggest the diagnosis of hepatic AL amyloidosis, thereby improving diagnostic accuracy and timely recognition among peers.

METHODS

We retrospectively analyzed the clinical, CT, and MRI findings of 10 patients with confirmed hepatic AL amyloidosis.

RESULTS

The predominant clinical manifestations included hepatomegaly (10/10, 100%), abdominal distension (9/10, 90%), fatigue (4/10, 40%), and weight loss (3/10, 30%). Laboratory findings showed markedly elevated alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) (10/10, 100%), with normal or mildly elevated transaminases; hypoalbuminemia was present in all patients (10/10, 100%). CT imaging demonstrated diffuse hepatomegaly with decreased hepatic attenuation in all 8 patients who underwent CT. MRI (6 patients) showed marked hepatomegaly with smooth margins, mildly and uniformly increased T2WI signal, along with vascular rarefaction. Contrast-enhanced CT/MRI revealed delayed enhancement. Additionally, poor visualization or stenosis of the main hepatic veins and periportal edema were also observed in all cases (10/10, 100%). MRCP was performed in 5 patients, and none showed evidence of biliary obstruction. Splenic hypoperfusion was observed in 7 patients, and renal hypoperfusion in 2 patients.

CONCLUSIONS

Despite its low incidence, hepatic AL amyloidosis should be suspected in patients presenting with unexplained hepatomegaly, markedly elevated ALP/GGT with relatively preserved transaminases, and specific imaging findings such as delayed enhancement, poor visualization or stenosis of the main hepatic veins, and periportal edema. Recognition of these clues, along with extrahepatic findings such as splenic or renal hypoperfusion, can significantly improve diagnostic accuracy and timely recognition of this disease.