Abe Y, Muta K, Ohshima K, Hirase N, Matsushima T, Yufu Y, Nishimura J, Nawata H
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Int J Hematol. 2000 Jun;71(4):379-84.
We describe a rare case of cytotoxic gastrointestinal T-cell lymphoma with protein-losing enteropathy. Initial examination revealed the coexistence of T-cell lymphoma and tuberculosis in the mesenteric lymph node and liver. Despite anti-tuberculosis and anti-cancer treatment, the patient experienced chronic diarrhea and malabsorption and died approximately 3 years after onset. Autopsy specimens revealed medium-sized lymphoma cells, with a phenotype of CD3+, CD4-, CD7+, CD8+, CD30-, CD56-, CD103 (HML-1)-, TIA-1+, and granzyme B+, proliferating primarily and consistently in the mucosa of the entire bowel tract from esophagus to rectum. Interestingly, Epstein-Barr virus (EBV)-encoded small nuclear RNAs were detected in the tumors by in situ hybridization. Southern blot analysis revealed monoclonal proliferation in the EBV-infected T cells. Although the present case can possibly be categorized as an intestinal T-cell lymphoma according to the Revised European-American Lymphoma Classification, the case showed a unique clinical course and distribution of lymphoma cells. We present here an interesting case of gastrointestinal cytotoxic T-cell lymphoma and examine the possible association with infectious agents.
我们报告一例罕见的伴有蛋白丢失性肠病的细胞毒性胃肠道T细胞淋巴瘤病例。初始检查发现肠系膜淋巴结和肝脏中同时存在T细胞淋巴瘤和结核病。尽管进行了抗结核和抗癌治疗,但患者仍经历慢性腹泻和吸收不良,并在发病后约3年死亡。尸检标本显示为中等大小的淋巴瘤细胞,其表型为CD3 +、CD4 -、CD7 +、CD8 +、CD30 -、CD56 -、CD103(HML - 1) -、TIA - 1 +和颗粒酶B +,主要且持续地在从食管到直肠的整个肠道黏膜中增殖。有趣的是,通过原位杂交在肿瘤中检测到爱泼斯坦 - 巴尔病毒(EBV)编码的小核RNA。Southern印迹分析显示EBV感染的T细胞呈单克隆增殖。尽管根据修订的欧美淋巴瘤分类,本病例可能归类为肠道T细胞淋巴瘤,但该病例显示出独特的临床病程和淋巴瘤细胞分布。我们在此呈现一例有趣的胃肠道细胞毒性T细胞淋巴瘤病例,并探讨其与感染因子的可能关联。