イレウスが診断の契機となった原発性アミロイドーシスの1例

A Case of systemic Primary Amyloidosis with Functional Ileus

　症例は55歳，女性．1995年4月頃より眩暈，失神が出現するようになった．同年7月当科受診し，軽度心不全，肥大型心筋症，洞不全症候群と診断された．初診時の心電図では低電位傾向肢誘導，V_1〜4でr波の減高と軽度ST上昇，II,III,aV_F，V_4〜6にて陰性T波を認め，心エコーでは心室中隔17mm，左室後壁14mmであった．同年8月上旬DDDペースメーカが植え込まれたが，同年11月心不全症状出現し再入院した．経過中イレウス，胆？炎なども併発したが保存的治療にて軽快した．イレウスが発症した時点で臨床経過，心エコー所見などによりアミロイドーシスを疑い直腸，胃生検を行ったところ本症と診断された．その後心不全症状は軽快せず，1996年2月2日心室頻拍よりペーシング不全となり死亡した．剖検所見では心重量510gで，心筋では筋層内，血管壁へ，肺では血管壁，肺胞壁ヘのアミロイド沈着があり，特に肺で沈着が高度であった．免疫組織化学的検索にてALアミロイドが検出され，本症は原発性アミロイドーシス（Aλ型）と診断した．

A 55-year-old woman was admitted to our hospital because of faintness and syncope. We diagnosed this case as congestive heart failure, hypertrophic car-diomyopathy with sick sinus syndrome (SSS). Electro-cardiogram revealed low voltage and poor r progression with ST-T changes. Transthoracic echocardiogram showed left ventricular hypertrophy. We evaluated the severity of SSS by electrophysiologic study. She was implanted DDD pacemaker. And then 3 months later, she was re-admitted to our hospital because of severe congestive heart failure. Chest X-ray revealed massive It. pleural effusion. 7 days after admission, she com-plained of abdominal pain and nausea due to ileus. She underwent conservative therapy for functional ileus. Rectal and gastric biopsy showed amyloid deposits in submucosa of the rectum and stomach. She was treated for cardiac failure, ileus and cholecystitis. However, she was died of heart failure and pacing failure. The autopsy revealed cardiomegaly (510g), microscopic examination showed amyloid deposits in the walls of the heart, and the vesicles and microvessel of the bilateral lung diffusely. The amyloid proteins were identified as AL (Aλ) type by immunohistochemical technology.