Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Pediatr Blood Cancer. 2010 Apr;54(4):610-2. doi: 10.1002/pbc.22330.
A rare case of primary intestinal T-cell lymphoma (ITL) of an 8-year-old boy is reported. Medium- to large-sized tumor cells were betaF1+, CD3+, CD8+. TIA-1+, but CD4-, CD5-, CD30-, CD56-, CD20-, CD79a-, TdT-, consistent with an intraepithelial lymphocyte (IEL) origin. They showed monoclonal rearrangement of the T-cell receptor gamma-chain and no evidence of EBV infection. No clinical, histologic, laboratory, or genetic evidence of celiac disease was detected. In adults, ITL is often associated with enteropathy and has a very poor outcome. Our patient remains in first remission 30 months after finishing the acute lymphoblastic leukemia protocol COALL-07-03 high risk standard.
报告一例 8 岁男童原发性肠道 T 细胞淋巴瘤（ITL）。中等至大肿瘤细胞βF1+、CD3+、CD8+。TIA-1+，但 CD4-、CD5-、CD30-、CD56-、CD20-、CD79a-、TdT-，符合上皮内淋巴细胞（IEL）起源。它们表现出 T 细胞受体 γ 链的单克隆重排，没有 EBV 感染的证据。未发现乳糜泻的临床、组织学、实验室或遗传证据。在成人中，ITL 常与肠病有关，预后非常差。我们的患者在完成急性淋巴细胞白血病协议 COALL-07-03 高危标准后 30 个月仍处于首次缓解期。
