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Nonsecreting Immunoproliferative Small Intestinal Disease: Report of a Case

非分泌性免疫增殖性小腸疾病:一病例報告

摘要


免疫增殖性小腸疾病好發於中東及地中海沿海地區,在台灣僅有少數病例被報告過。此病患者臨床上常以腹瀉、吸收不良、和體重減輕來表現,診斷有賴於小腸病理切片檢查以及在血清或組織體液中發現甲種重鏈蛋白質。我們報告一例具典型臨床症狀的31歲男性患者,其空腸切片檢查看到黏膜固有層中有緻密的淋巴漿細胞浸潤,浸潤程度已穿越黏膜肌肉層。但在病人的血清、尿液、十二指腸液、和空腸切片組織中無法顯示出甲種重鏈蛋白質。其病程分期屬於前淋巴瘤期的晚期。治療方面,病人拒絕接受化學治療,故給予嘗試性口服抗生素(四環黴素)療法。數星期後,臨床症狀改善。四個月後,臨床症狀完全消失,各項檢驗數據亦恢複正常,病人因而自行停藥。八個月時,重覆小腸鏡追蹤及空腸切片檢查仍舊可以看到黏膜腫脹和淋巴球浸潤的現象,所以病人重新接受口服四環黴素治療。十個月後,病人發生全身性淋巴腺腫大,頭部淋巴結及空腸切片檢查證實為惡性淋巴瘤。此後病人接受七次複合式化學治療而得到完全緩解。現今病人仍繼續於門診密切追蹤中。

並列摘要


Immunoproliferative small intestinal disease has rarely been reported in Taiwan. A 31-year-old man had suffered from severe diarrhea and malabsorption for one year prior to admission. Jejunal biopsy through enteroscopic examination revealed atrophic villi and dense infiltration in lamina propria by lymphocytes, with extension beyond the layer of muscularis mucosa. Alpha-chain protein was not demonstrated in the patient's serum, urine, diluted duodenal Juice, and jejunal biopsy tissue. He was treated with metronidazole for three weeks per os and tetracycline for four months. Frequency of diarrhea decreased two weeks after treatment. Clinical symptoms and signs of malabsorption improved gradually. However, lymphadenopathies developed on both nuchal, axillary, and inguinal areas after a ten-month symptom-free period. The diagnosis of non-Hodgkin's lymphoma, diffuse large B cell type, stage щ a, was made following systemic work-up. Systemic chemotherapy was administered with complete clinical remission. To our knowledge, this is the first case of nonsecreting immunoproliferative small intestinal disease reported in Taiwan. Furthermore, immunoproliferative small intestinal disease should be considered in the differential diagnosis of longterm diarrhea, especially in the presence of malabsorption.

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