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肠病相关T细胞淋巴瘤:一例病例报告及文献综述

Enterotherapy associated T-cell lymphoma: a case report and literature review.

作者信息

Dieter R S, Duque K

机构信息

University of Wisconsin Hospital, Chicago, IL, USA.

出版信息

WMJ. 2000 Oct;99(7):28-31.

Abstract

UNLABELLED

T-cell lymphoma of the intestine is a form of extranodal, non-Hodgkins lymphoma. Enteropathy associated T-cell lymphoma (EATCL) is a unique form of T-cell lymphoma involving the gastrointestinal tract. This disorder is rare and there is no consensus on guidelines for diagnosis or treatment.

CASE

This 47 year old patient presented to the hospital with increasing abdominal girth and swelling of the lower extremities over the four days prior to admission. He complained of dyspnea on exertion, anorexia, nausea and vomiting, 40 pound weight loss over half a year, accompanied by intermittent diarrhea. On physical examination, the patient was found to have anasarca, an abdominal fluid wave with shifting dullness, mild abdominal tenderness, marked pitting edema of the extremities, and necrotic toes. A small bowel follow through revealed an abnormality in the mid-jejunum and a colonoscopy revealed two erythematous lesions near the cecum. Subsequent esophagoduodenoscopy and enteroscopy identified multiple white patchy lesions in the proximal jejunum. Biopsies were consistent with a T-cell lymphoma. The patient underwent a partial small bowel resection. Biopsies of the jejunum had ulceration and necrosis with a lymphomatous infiltrate. The lymphocytes were found to be a monoclonal CD2 positive T-cell population. A bone marrow biopsy was unremarkable. Enteropathy associated T-cell lymphoma is a rare, and this may be the first reported case of EATCL presenting as anasarca.

DISCUSSION

The etiology and pathophysiology of intestinal lymphomas, particularly enteropathy associated T-cell lymphoma, is reviewed.

CONCLUSION

EATCL is a rare and unique form of lymphoma without an exact treatment regimen which carries a high mortality rate.

摘要

未标注

肠道T细胞淋巴瘤是一种结外非霍奇金淋巴瘤的形式。肠病相关T细胞淋巴瘤(EATCL)是累及胃肠道的一种独特的T细胞淋巴瘤形式。这种疾病很罕见,对于其诊断或治疗指南尚无共识。

病例

该47岁患者在入院前四天因腹围增大和下肢肿胀入院。他主诉活动时呼吸困难、厌食、恶心和呕吐,半年内体重减轻40磅,伴有间歇性腹泻。体格检查发现患者有全身性水肿、腹部液波震颤及移动性浊音、轻度腹部压痛、双下肢明显凹陷性水肿和脚趾坏死。小肠造影显示空肠中段异常,结肠镜检查显示盲肠附近有两个红斑性病变。随后的食管十二指肠镜检查和小肠镜检查发现空肠近端有多个白色斑片状病变。活检结果符合T细胞淋巴瘤。患者接受了部分小肠切除术。空肠活检有溃疡和坏死,并伴有淋巴瘤浸润。发现淋巴细胞为单克隆CD2阳性T细胞群体。骨髓活检无异常。肠病相关T细胞淋巴瘤很罕见,这可能是首例以全身性水肿为表现的EATCL病例。

讨论

对肠道淋巴瘤,尤其是肠病相关T细胞淋巴瘤的病因和病理生理学进行了综述。

结论

EATCL是一种罕见且独特的淋巴瘤形式,没有确切的治疗方案,死亡率很高。

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