Chronic intestinal pseudo-obstruction due to dialysis-related amyloid deposition in the propria muscularis in a hemodialysis patient

Dialysis-related amyloidosis (DRA) is one of the most serious complications interfering with rehabilitation in dialysis patients. Here, we report a ca se of beta(2)-microglobulin (beta(2)M)-related amyloidosis, in which the pa tient developed a severe intestinal pseudo-obstruction. The patient was a 4 2-year-old male who had been undergoing hemodialysis for 13 years, and who had no history of osteoarticular involvement of DRA. The first symptoms of the disease were severe abdominal fullness and nausea after meals. The whol e intestinal wall biopsy revealed massive amyloid deposition in the propria muscularis. The patient became malnourished and died of acute subendocardi al infarction 3 years after the onset. An autopsical examination revealed a massive deposition of amyloid, which was positively stained with anti- bet a(2)M antibody but not AA amyloid, predominantly in the gastrointestinal mu scular layer, including the tongue, esophagus, stomach, small intestines, c olon, and rectum. These results suggest that the gastrointestinal involveme nt of beta(2)M-relaled amyloidosis might occur during the course of hemodia lysis treatment, and that this possibility should be considered if patients suffer from intestinal pseudo-obstruction without osteoartricular symptoms .