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肠病相关T细胞淋巴瘤

[Enteropathy associated T-cell lymphoma].

作者信息

Bakrac Milena, Bonaci-Nikolić Branka, Colović Natasa, Simić-Ogrizović Sanja, Krstić Miodrag, Colović Milica

出版信息

Srp Arh Celok Lek. 2007 Jan-Feb;135(1-2):80-4. doi: 10.2298/sarh0702080b.

DOI:10.2298/sarh0702080b
PMID:17503573
Abstract

Enteropathy associated T-cell lymphoma (EATCL) is a high grade, pleomorphic peripheral T-cell lymphoma with usually cytotoxic phenotype. This is a case report of three patients with EATCL. The first patient was 50 year-old woman with four-year history of gluten sensitive enteropathy (GSE). Diagnosis of lymphoma was confirmed after the resection of the jejunum (small intestine obstruction). Pathohistological (PAS, Reticulin, Giemsa) and immunohistochemical (anti-LCA, anti-CD20, anti-CD45RO, anti-CD3) methods revealed the diagnosis of EATCL: CD45RO+, CD3+. After the third cycle of chemotherapy, the disease progressed with massive lung infiltration. Patient died due to complications of bone marrow aplasia. The second patient was 23 year-old woman with long earlier history of GSE. She presented with the acute renal failure. According to established diagnosis of tubulointerstitial nephritis, she was treated with pulse doses of steroid therapy. After temporary improvement, she had dissemination of the disease. On MRI, small intestinal wall was thickened, and abdominal lymph nodes were enlarged with extraluminal compression of common bile duct. Laparotomy with mesenterial lymph node biopsy and consecutive pathohistological and immunohistochemical analyses revealed the diagnosis of EATCL. The patient received chemotherapy, but she died with signs of pulmonary embolization. The third patient was 53 year-old woman without previous history of GSE. Diagnosis of EATCL was revealed after the resection of jejunum because of small intestinal obstruction. She received two cycles of chemotherapy, but she died with signs of disease progression. IgA antiendomysial antibodies were detected in the serum of all patients. The overall survival of patients was 7 months.The possibility of lymphoma rising in patients with clinical progression of GSE despite gluten free diet must be kept in mind.

摘要

肠病相关T细胞淋巴瘤(EATCL)是一种高级别、多形性外周T细胞淋巴瘤,通常具有细胞毒性表型。本文报告3例EATCL患者。首例患者为一名50岁女性,有4年麸质敏感性肠病(GSE)病史。空肠切除术后(因小肠梗阻)确诊为淋巴瘤。病理组织学(PAS、网状纤维、吉姆萨)和免疫组织化学(抗LCA、抗CD20、抗CD45RO、抗CD3)方法确诊为EATCL:CD45RO+、CD3+。化疗第3周期后,疾病进展,出现大量肺部浸润。患者因骨髓再生障碍并发症死亡。第二例患者为一名23岁女性,有较早的GSE病史。她出现急性肾衰竭。根据确诊的肾小管间质性肾炎,给予大剂量脉冲式类固醇治疗。病情暂时改善后,疾病出现播散。MRI显示小肠壁增厚,腹部淋巴结肿大,压迫胆总管腔外。剖腹探查并行肠系膜淋巴结活检,随后的病理组织学和免疫组织化学分析确诊为EATCL。患者接受化疗,但死于肺栓塞征象。第三例患者为一名53岁女性,既往无GSE病史。因小肠梗阻行空肠切除术后确诊为EATCL。她接受了2周期化疗,但死于疾病进展征象。所有患者血清中均检测到IgA抗肌内膜抗体。患者总生存期为7个月。必须牢记,即使采用无麸质饮食,GSE临床进展的患者仍有发生淋巴瘤的可能。

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[Enteropathy associated T-cell lymphoma].肠病相关T细胞淋巴瘤
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[Acute intestinal obstruction revealing enteropathy associated t-cell lymphoma, about a case].[急性肠梗阻揭示肠病相关T细胞淋巴瘤,一例报告]
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