Mutations in the fibrinogen Aα-chain genes are the most common cause of 
hereditary renal amyloidosis. The renal histologic appearance in the patient is 
characteristic and shows striking glomerular enlargement with almost complete 
obliteration of the normal glomerular architecture by extensive amyloid 
deposition. In contrast, the vessels and renal tubular interstitium of such 
patient contains almost no amyloid at all. Here, we described a patient with 
hereditary fibrinogen amyloidosis, who presented with proteinuria, hypertension 
and renal failure. He was shown to be heterozygous for the relevant mutation 
encoding the E526V fibrinogen variant.