Primary amyloidosis presenting with cholestasis and hyperkinetic portal hypertension.

Amyloidosis is not a single disease, but a series of diseases in which there is extracellular deposition of a protein in an abnormal fibrillar form. We report a 48-year old woman with subicteric coloration of scleras and hepatomegaly. Functional liver tests evidenced a high level of alkaline phosphatase and serum gamma-glutamyl transpeptidase with mild increase of bilirubin level. Color Doppler ultra-sonography, showed a hyperkinetic portal hypertension. The liver biopsy found amyloid in sinusoids subendothelially. Immunohistochemical staining for anti lambda light chain immunoglobulin was positive. Bone marrow morphology and immunohistochemistry confirmed lymphoplasmocytic lymphoma with amyloidosis.