[Sézary syndrome associated with small intestinal amyloidosis]

A 68-year-old female diagnosed as having Sézary syndrome because of generalized erythroderma and increased lymphocytes with convoluted nuclei in 1988 was treated with steroids. However she did not respond to therapy and died due to pneumonia and aggravation of Sézary syndrome in September, 1994. Dyspeptic diarrhea was observed for two months before she died. Autopsy revealed local deposition of amyloid A protein in the mucosa of the small intestine, which might have been the cause of dyspeptic diarrhea. This was a rare case which Sézary syndrome accompanied by localized amyloidosis.