Initial Diagnosis of Unsuspected Primary Amyloidosis (AL) by Liver Biopsy

Abstract

While the liver is commonly involved in systemic amyloidosis, review of the literature indicates that clinically evident hepatic amyloidosis is not usually encountered. Three patients with clinically unsuspected primary amyloid light chain (AL) type of amyloid osis diagnosed by liver biopsy are described. All patients presented with signs, symp toms, and biochemical abnormalities consistent with intrinsic liver disease. The first patient had cholestatic hepatitis with jaundice, the second had hepatomegaly and weight loss, and the third had cholestatic liver disease. All three liver biopsies showed extensive sinusoidal deposition of amyloid material that stained positively with Congo red with and without prior potassium permanganate digestion, consistent with AL type amyloid. Ultrastructural examination of one of the cases showed straight, non- branching fibrils having the characteristic appearance of amyloid. Immunocytochemi cal staining in another case revealed the hyaline material to be positive for lambda light chains and negative for kappa light chains. These cases demonstrate that primary amyloidosis can present with liver abnormalities that dominate the clinical picture and that liver biopsy may be the initial diagnostic procedure of unsuspected primary amyloidosis. Int J Surg Pathol 1 (1): 57-64, 1993