中华皮肤科杂志

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儿童皮下脂膜炎样T细胞淋巴瘤的研究

万川1, 王琳2, 张文燕3, 唐源3, 徐晨2, 李薇2, 李甘地3, 刘卫平3   

  1. 1. 江西省皮肤病专科医院, 330001;
    2. 四川大学华西医院皮肤性病科, 成都, 610041;
    3. 四川大学华西医院病理科, 成都, 610041
  • 收稿日期:2006-11-08 出版日期:2007-10-15 发布日期:2007-10-15
  • 通讯作者: 王琳,email:LKZWL@126.com E-mail:LKZWL@126.com
  • 基金资助:
    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)

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

WAN Chuan1, WANG Lin2, ZHANG Wen-yan3, TANG Yuan3, XU Chen2, LI Wei2, LI Gan-di3, LIU Wei-ping3   

  1. Department of Dermatovenereology, West China Hospital, Sichuan University, Chengdu 610041, China
  • Received:2006-11-08 Online:2007-10-15 Published:2007-10-15

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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。

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

Abstract: 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 clinico-pathologically.Immunohistochemical staining forβF1,CD2,CD3,CD45RO,CD4,CD8,CD20,CD30, CD56,TIA-1 and granzyme B were performed.PCR was used to assess the rearrangement of TCRγand 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 expressedβF1,CD2, CD3,CD8,CD45RO,TIA-1 and granzyme B,but did not express CD4 or CD20.Monoclonal TCRγgene 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.

Key words: Children, Lymphoma, T-cell, Panniculitis, Herpesvirus 4, human, Gene rearrangement