结肠ALK阴性的间变性大细胞淋巴瘤1例报道并文献复习

ALK- negative colonic anaplastic large cell lymphoma: a case report with clinicopathologic analysis and literature review

目的:探讨肠道原发性间变性淋巴瘤激酶(ALK)阴性的间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALK-negative,ALK-ALCL)的临床病理学特点、诊断及鉴别诊断。方法:收集1例结肠原发性ALK-ALCL病理标本,对其进行临床病理分析、免疫组织化学检测及EB病毒编码的RNA(EBER)原位杂交检测并复习相关文献。结果:患者,男,35岁,因"间断解血便5年,腹痛2个月,发热1个月"入院。临床长期以肠结核、炎症性肠病(克罗恩病)治疗,一度有效。近一个月来,因反复发作高热,临床考虑肠穿孔,剖腹探查后行结肠次全切术。大体观察:结肠内可见直径3.5-9.0cm的溃疡3个,最大溃疡处可见穿孔。光镜下肿瘤组织弥漫浸润肠壁全层及其周围脂肪组织,可见较多"标志性"肿瘤细胞,形态符合普通型ALCL。免疫组织化学示肿瘤细胞弥漫表达CD30,表达T细胞抗原CD3,表达细胞毒性抗原穿孔素,ALK阴性。EBER原位杂交检测为阴性。结论:肠道原发ALK-ALCL是罕见的结外T细胞淋巴瘤,恶性度高,但因临床症状不具备特征性易导致临床误诊。诊断依据病理学形态特点及免疫组织化学指标综合判断。

Objective：To report the clinicopathological characteristics,diagnosis and differential diagnosis of intes-tinal anaplastic large cell lymphoma,ALK-negative（ ALK-ALCL）. Methods：The clinical features,histopathologi-cal changes,immunohistochemical findings and Epstein-Barr virus-encoded RNA（ EBER）were analyzed on a case of ALK-ALCL with review of the related literature. Results：A 35-year-old man developed discontinuous bloody diarrhea 5 years,abdominal pain 2 months,and fever 1 month before admission. The anti-tuberculosis and inflamma-tory bowel disease（ crohn＇s disease）therapies were then initiated with relief of symptoms. A month later,however,the patient complained of repeated fever and bloody diarrhea. He was clinically suspected with bowel perforation and un-derwent subtotal colostomy. Multifocal ulcers in the colon with 3. 5-9. 0cm in diameter were observed,and the biggest with perforation. Microscopic examination of the colon revealed an extensive infiltration of full-thickness of intestinal wall and extended into the adipose tissue. The tumor was classified as the〞common pattern〞 ALCL. Tumor cells ex-pressed CD30,and showed evidence of T-cell line age with cytotoxic potential by expression of CD3 and perforin. Anaplastic lymphoma kinase（ ALK）was negative,and in situ hybridization for EBER negative. Conclusion：Extranod-al T cell lymphoma,primarily involving intestine is rare and tends to carry an aggressive clinical course. The relatively non-specific clinical manifestations of intestinal ALK-ALCL may result in misdiagnosis in some cases. A compre-hensive evaluation of clinical manifestations,pathologic features and immunohistochemical findings is essential for di-agnosis.