以假性腸道阻塞為起始表現的類澱粉沉積症：一病例報告

假性腸道阻塞與許多疾病都有關聯，併發於腸道類澱粉沉積症的病例較罕見，我們報告一例多發性骨髓瘤合併類澱粉沉積症並以假性腸道阻塞為初始表現的病例。病人為一42歲女性，主訴腹漲及間歇性血便有一個月，系列的影像學檢查均懷疑在末端迴腸處有病灶，而引起腸道阻塞，並在懷疑腸道淋巴瘤的診斷下接受剖腹探查，術中發現從Treitz韌帶到迴盲瓣有廣泛性腸壁增厚，且有腸系膜淋巴結腫大，病理組織檢查可見有大量類澱粉沉積在黏膜下層、血管壁內及肌肉層，其後之骨髓穿刺及血清免疫檢查證實為多發性骨髓瘤，後雖積極的施予化學治療，但是病人仍在診斷後三個月因大量上消化道出血而死亡。此病例提醒臨床醫師，對假性腸道阻塞的病人，在病因的探討上，類澱粉沉積應列為鑑別診斷的考慮。

關鍵字

類澱粉沉積症；假性腸道阻塞

並列摘要

Intestinal pseudo-obstruction has been described in association with a large number of disease entities. It is rare in intestinal amyloidosis complicating intestinal pseudo- obstruction. We report a case of multiple myeloma associated with intestinal amyloidosis with the initial presentation of intestinal pseudo-obstruction. A 42-year-old woman suffered from intermittent abdominal distention and bloody stool passage for one month. After a series of examinations, terminal ileal lesions associated with partial intestinal obstruction was diagnosed. Exploratory laparotomy was performed under the impression of intestinal lymphoma. Operative findings revealed increased thickness of intestinal wall diffusely from Treitz ligament to terminal ileum and enlarged mesenteric lymph nodes. Histopathologic features revealed massive amyloid deposition in intestinal vascular wall, submucosa and muscular propria. Multiple myeloma was confirmed thereafter by bone marrow biopsy and immunologic study of serum. She received 4 courses of chemotherapy with limited effect. Unfortunately, she died due to massive upper gastrointestinal tract bleeding 3 months after initial diagnosis. In view of intestinal pseudo-obstruction, we suggest that intestinal amyloidosis be kept in mind in the list of differential diagnoses.

並列關鍵字

amyloidosis ； intestinal pseudo-obstruction

國際替代計量

以假性腸道阻塞為起始表現的類澱粉沉積症：一病例報告

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