Bakrac Milena, Bonaci Branka, Krstic Miodrag, Simic Sanja, Colovic Milica
Institute of Hematology, Clinical Center of Serbia, Koste Todorovica 2, 11000 Belgrade, Serbia.
World J Gastroenterol. 2006 Apr 14;12(14):2301-4. doi: 10.3748/wjg.v12.i14.2301.
Enteropathy-associated T-cell lymphoma (EATCL) is a high grade, pleomorphic peripheral T-cell lymphoma usually with cytotoxic phenotypes. We describe a first case of patient with EATCL that is remarkable for its fulminant course and invasion of both kidneys manifested as acute renal failure. The patient was a 23 year old woman with a long history of celiac disease. She was presented with acute renal failure and enlarged mononuclear infiltrated kidneys. Diagnosis of tubulointerstitial nephritis and polyserositis was confirmed with consecutive pulse doses of steroid therapy. After recovery, she had disseminated disease two months later. Magnetic resonance imaging showed thickened intestine wall, extremely augmented kidneys, enlarged intra-abdominal lymph nodes with extra-luminal compression of common bile duct. Laparotomy with mesenterial adipous tissue and lymph glands biopsy was done. Consecutive pathophysiological and immunohistochemical analyses confirmed the diagnosis of EATCL: CD45RO+, CD43+, CD3+. The revision of renal pathophysiology substantiated the diagnosis. The patient received chemotherapy, but unfortunately she died manifesting signs of pulmonary embolism caused by tumor cells.
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。我们描述了首例EATCL患者,其病程凶险,双侧肾脏均受侵犯,表现为急性肾衰竭。该患者为一名23岁女性,有长期乳糜泻病史。她因急性肾衰竭和单核细胞浸润性肾脏肿大就诊。连续静脉注射类固醇治疗确诊为肾小管间质性肾炎和多浆膜炎。康复后,两个月后她出现了播散性疾病。磁共振成像显示肠壁增厚、肾脏极度增大、腹腔内淋巴结肿大并对胆总管造成管腔外压迫。进行了剖腹手术并取肠系膜脂肪组织和淋巴结活检。连续的病理生理和免疫组化分析确诊为EATCL:CD45RO+、CD43+、CD3+。肾脏病理生理学检查结果进一步证实了诊断。患者接受了化疗,但不幸的是,她因肿瘤细胞导致的肺栓塞迹象而死亡。