monoclonal gammopathyを伴い胃と腸に限局すると思われるアミロイドーシスの1例

Gastrointestinal Amyloidosis, Report of a Case

要旨　患者は69歳の女性で貧血の精査目的で1984年11月に入院．1981年に胃切歴あり．血清M蛋白と尿中Bence Jones蛋白を微量検出したが骨髄は異常なし．X線・内視鏡検査にて残胃全域に大小の不整形陥凹と小結節状隆起を認め，小腸では蠕動低下とKerckring皺襞の腫大，点状小陥凹を，大腸では毛細血管像が消失し褪色した粘膜に小陥凹を認めた．胃と直腸生検でアミロイド沈着を認めた．消化管出血が遷延し残胃全摘と空腸部分切除，肝脾の楔状切除を実施．病理学的に残胃と小腸の粘膜筋板から粘膜下層に著明なアミロイド沈着があるも，食道にはEC結合部近傍にごく微量認めるのみで，肝脾に同沈着はなかった．なお，1981年の遡及的資料も呈示した．

　A 69-year-old woman was admitted to our hospital for further examination of anemia on Nov. 2, 1984. She had a past history of subtotal gastrectomy in Sep. 1981. Familial history was not significant. A very small quantity of serum M-protein and urinary Bence Jones protein were detected, but bone marrow examination did not show plasmacytosis. Other laboratory data were unremarkable.

　X-ray and endoscopic examinations in Dec. 1984 revealed many irregularly-shaped erosions and ulcerations surrounded by nodular shadows in the whole remnant stomach, and barium flecks of spotty or larger size within the hyperlucent shadows in the duodenum. Diminished peristalsis, edematously thickened Kerckring's folds, numerous tiny barium flecks, and coarse mucosal appearance were seen in the small intestine. Large intestine showed slightly irregular narrowing, coarse and discolored mucosal appearance without normal vascular network, and shallow depressions.

　Biopsy specimens obtained from the stomach and rectum showed amyloid deposits.

　The entire remnant stomach and part of jejunum were resected on Dec. 17, 1984. Wedge resections of the liver and spleen were performed. Pathologically, there was an extensive amyloid deposition in the muscularis mucosae and submucosae of the resected stomach and ileum. Amyloid deposit was noticeable in the region around the small vessels in the submucosa. The esophagus was also deposited by a very small amount of amyloid at the EC junction, but the resected liver and spleen were negative for amyloid deposit.

　Retrospectively, endoscopic examination on Jan. 10, 1981 had shown coarse and discolored mucosa distal to the lower corpus, and slightly depressed area at the antrum. Endoscopic examination on Aug. 24, 1981 (three days after the onset of hematemesis) had also shown two ulcerative lesions surrounded by nodular elevations in the antrum. Subtotal gastrectomy was then performed on Sep. 3, 1981. Pathological re-study revealed a pronounced amount of amyloid deposit near the basis of the ulcers, and small amount in the muscularis mucosae proximal to the oral end of the resected stomach.