儿童皮下脂膜炎样T细胞淋巴瘤1例报道并文献复习

Case Report and Review of Literature of Child Subcutaneous Panniculitis-Like T-Cell Lymphoma

目的分析儿童皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床表现及病理组织学特征,探讨其病理诊断及鉴别诊断,提高儿科医师对该病的认识。方法反复发热伴多发性皮肤损害、病程长达2a确诊的SPTCL患儿1例,对其临床表现的演变、多次被误诊的就医经历、确诊时的组织病理学特点、免疫组织化学结果及T细胞受体(TCR)基因克隆重排等方面进行观察,并对相关文献进行复习。结果皮肤活检发现组织学病变主要局限在皮下脂肪组织内,非典型淋巴细胞围绕单个脂肪细胞形成特征性的花边样结构。免疫组织化学显示CD45、CD45RO、CD3、CD5、T细胞内抗原-1及穿孔素呈阳性表达,而CD10、CD20、CD56、CD68、上皮膜抗原和细胞角蛋白均呈阴性,提示瘤细胞为T细胞表型。TCR基因克隆重排检测:免疫球蛋白H基因重排FR2(+),FR3A(-);TCRγ基因重排JVI(-),JVII(+);TCRβ基因重排JD1(-),JD2(-)。按照儿童T细胞非霍奇金淋巴瘤方案治疗,但疗效欠佳。结论SPTCL是一类特殊类型的原发性皮肤淋巴瘤,预后较差,对不明原因的反复发热伴皮肤多发性损害的儿童,病损处皮肤活检是确诊SPTCL的重要手段。

Objective To analyze the clinical and histopathologic characteristics in children with subcutaneous panniculitis - like T - cell lymphoma（SPTCL）, and explore the pathological diagnosis and differential diagnosis, in order to boost paediatricians to better understanding the disease. Methods One case was diagnosed SVFCL with over 2 years protracted course of fever and muhiple skin lesions. The evolvement of clinical presentation, the misdiagnosed experience, the histopathologieal features, the immunohistochemical results and T cell receptor （TCR） gene cloning rearrangement were observed. The related literatures published were reviewed. Results Skin biopsy showed that the histopathologic findings were limited within subcutaneous fatty tissue, the atypical lymphocytes characteristically rimmed individual fat cells in a lace - like pattern. Immunophenotypie studies showed CD45, CD45 RO, CD3, CD5, T cell intracellular antigen - 1 （ TIA - 1 ） and perfofin usually expressed possitive, while CD10 ,CD20 ,CD56, CD68, epithelial membrane antigen（EMA） and cytokeratin （CK） were negative, implying tumor cells derived from T - cell. The results of TCR gene rearrangement as following： IgH FR2 （ ＋ ） , FR3A （ - ） ;TCR γ JVI （ - ） ,JVII（ ＋ ） ; TCRβ JD1 （ - ）, JD2（ - ）. Although the protocol of children＇s T - cell Non - Hodgkin lymphoma was administrated, the treatment outcome was poor. Conclusion SPTCL is a special primary cutaneous lymphoma with poor prognosis, skin biopsy of suffered lesion is an important method for the diagnosis of SPTCL children with unclear recurrent fever and muhiple skin lesions.