儿童皮下脂膜炎样T细胞淋巴瘤的研究  

A study of subcutaneous panniculitis-like T-cell lymphoma in children

作　　者：万川[1] 王琳[1] 张文燕[2] 唐源[2] 徐晨[1] 李薇[1] 李甘地[2] 刘卫平[2] 

机构地区：[1]四川大学华西医院皮肤性病科 [2]四川大学华西医院病理科,成都610041

出　　处：《中华皮肤科杂志》2007年第10期594-597,共4页Chinese Journal of Dermatology

基　　金：2004年四川大学青年科学研究基金(校200350);2005年四川大学华西医院留学回国人员科研启动基金(136050122)

摘　　要：目的探讨儿童皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床病理、免疫表型、基因重排及EB病毒(EBV)感染情况。方法将收集的5例儿童SPTL作临床病理和免疫组化分析,采用PCR检测TCRγ、IgH基因重排,运用EBER1/2原位杂交检测EBV感染。结果男4例、女1例,年龄9～13岁,主要表现为无症状的结节、斑块或肿块。组织学上肿瘤在皮下脂肪内呈脂膜炎样浸润,细胞大小不等、异形性明显,瘤内可见豆袋细胞、上皮样肉芽肿和小片状坏死。瘤细胞表达T细胞标记βF1、CD2、CD3、CD8、CD45RO和细胞毒颗粒相关蛋白标记TIA-1、粒酶B,不表达CD4、CD20。4例检出单克隆性TCRγ基因重排,未检出IgH基因重排。EBER1/2原位杂交阳性2例。获随访的3例中1例死于本病,该例EBER1/2阳性。结论儿童SPTL面部易受累,全身症状多见,噬血细胞综合征发生率与死亡率较高。伴有EBV感染者可能预后较差。Objective To investigate the clinicopathologic features and immunohistochemical profiles of, as well as gene rearrangement and Epstein-Barr virus （ EBV ） infection in subcutaneous panniculitis-like T-cell lymphoma （ SPTL ） in children. Methods Five cases of SPTL in children were analyzed clinicopathologically. Immunohistochemical staining for β F1, CD2, CD3, CD45RO, CD4, CD8, CD20, CD30, CD56, TIA-1 and granzyme B were performed. PCR was used to assess the rearrangement of TCRγ and IgH genes. The EBV infection was detected by in situ hybridization with EBER1/2. Results There were 4 male patients and 1 female patient, ranging in age from 9 to 14 years. The main clinical manifestations were asymptomatic nodules, plaques and masses. Histologically, a panniculitis-like infiltration was observed in the subcutaneous fat; the infiltrating cells varied in size and showed obvious atypia; there were beanbag cells, epithelioid granulomas and lamellar necrosis in the tumor tissues. These tumor cells expressed 13F1, CD2, CD3, CD8, CD45RO, TIA-1 and granzyme B, but did not express CD4 or CD20. Monoclonal TCRγ gene rearrangement was found in 4 of the 5 cases, while IgH gene rearrangement in none of them. EBV infection was found in 2 cases. Of the three patients available for follow up evaluation, one, who was positive for EBR1/2 died of SPTL. Conclusions In SPTL in children, face is apt to be involved, systemic findings are commonly seen, and hemophagocytic syndrome and death occur at a rather high rate. The prognosis may be poor in SPTL in children associated with EBV infection.

关 键 词：儿童 淋巴瘤 T细胞 脂膜炎 疱疹病毒4型 人 基因重排 

分 类 号：R739.5[医药卫生—肿瘤]