Hepatic amyloidosis with light chain deposition disease. A rare association

Monoclonal immunoglobulin deposition diseases are due to pathological prote in deposition in various tissues and organs. Protein deposits may be found in a single tissue or systemically and the organs most frequently involved are kidney, heart, peripheral nerves and the liver. Depending on the patter n of the deposits and the type of immunoglobulin, these diseases are distin guished as primary amyloidosis, light chain deposition disease. Differentia l diagnosis is made in tissue specimens: microscopically by the identificat ion of positive Congo red staining of the deposits, by immunohistochemical demonstration of proteins reacting with light chain (lambda or k) antisera or by recognition of fibrillar structures on electron microscopy. We report an unusual case of light chain deposition disease associated with amyloido sis, where hepatomegaly was the presenting manifestation and liver failure the cause of death, without any kidney involvement.