Idiopathic AL-kiv amyloidosis presenting as giant hepatomegaly

The 11/2-yr course of idiopathic systemic amyloidosis in a 63-yr-old woman was characterized by inanition, subcutaneous ecchymoses and giant hepatomegaly, the liver weighing 8.5 kg at autopsy. Skeletal survey and bone marrow aspirate were normal. The major components of isolated amyloid fibrils were 16,000- and 23,000-dalton proteins. The 16,000-dalton component was shown by amino acid sequencing to be a fragment of the kappa (k)iv light chain, the first such case. These clinicochemical correlations suggest that isolated massive hepatomegaly may prove to be a hallmark of idiopathic amyloid light chain-related protein k amyloidosis.