十二指腸下行部に潰瘍病変を伴ったアミロイドーシスの1例

Amyloidosis with Ulcerations of Descending Portion of the Duodenum, Report of a Case

要旨　患者は70歳の男性で，心窩部痛，タール便を主訴として入院．X線および内視鏡検査で十二指腸下行部の乳頭部近傍に出血性の陥凹性病変を認め，十二指腸癌を疑ったが，生検組織にアミロイド沈着を認めた．十二指腸出血が遷延し，X線および内視鏡検査で病変の伸展性があり，悪性リンパ腫も否定しえないため，膵頭十二指腸切除，胆囊摘除，小腸切除を行った．十二指腸下行部乳頭部の外側に2個の潰瘍，口側に線状瘢痕を認め，十二指腸全体に小血腫が散在性に多数認められた．切除小腸に3か所血腫を認めたが，胆囊，胃，膵に著変を見なかった．組織所見は十二指腸粘膜下層に広範なアミロイドの沈着と出血を認め，Ul-Ⅱの潰瘍と癩痕を伴っていた．高度のアミロイド沈着は十二指腸のみでなく，小腸の粘膜下層にも認めたが，胃，胆嚢，膵の血管壁にも認められた．アミロイド蛋白はAL型を示した．アミロイドーシスの上部消化管病変で，胃，小腸の切除報告はあるが，十二指腸病変の切除例は本例が初めてと思われる。膵頭十二指腸切除により，十二指腸のみでなく，他臓器にもアミロイド沈着があり，全身性アミロイドーシスと診断しえた．

　The patient was a 70-year-old man with the complaint of epigastralgia and tarry stool and found an ulcerative lesion with bleeding on the descending portion near the papilla of the duodenum. He was diagnosed as having suspected duodenal cancer. However, the biopsy specimen showed amyloid deposits. For continuous bleeding from the duodenal ulceration, pancreato-duodenectomy, cholecystectomy and resection of the small intestine was performed on Feb. 27, 1998. Pathologically, the entire resected duodenum showed massive amyloid deposits and bleeding on the submucosa with Ul-Ⅱ ulcers and scars, and slight amyloid deposits on the proper muscle and mucosa. The resected small intestine was 12 cm in length, and also showed localized massive amyloid deposits on the submucosa. Amyloid deposits were found on the vessel wall of the stomach, gallbladder and pancreas. This case of amyloid protein type was AL type.

　There are reports on amyloidosis in the upper gastrointestinal area, the stomach and the small intestine, but this is the first case report of the resection of the duodenum due to amyloidosis. After pancreato-duode-nectomy, amyloid deposits were detected not only in the duodenum, but also in several other organs, so the diagnosis was systemic amyloidosis.