| 儿童皮下脂膜炎样T细胞淋巴瘤1例报道并文献复习 |
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| 引用本文： | 袁晓军,张勤,姚晓红,王珍,盛琦. 儿童皮下脂膜炎样T细胞淋巴瘤1例报道并文献复习[J]. 实用儿科临床杂志, 2008, 23(24) |
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| 作者姓名： | 袁晓军 张勤 姚晓红 王珍 盛琦 |
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| 作者单位： | 1. 上海交通大学医学院附属新华医院儿内科,上海,200092
2. 上海交通大学医学院附属新华医院病理科,上海,200092 |
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| 摘 要： | 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2 a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-l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(-);TCRr基因重排JVI(-),JVII(+);TCRB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.
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| 关 键 词： | 淋巴瘤 皮肤 T细胞 脂膜炎 儿童 |
Case Report and Review of Literature of Child Subcutaneous Panniculitis-Like T-Cell Lymphoma |
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| YUAN Xiao-juo,ZHANG Qin,YAO Xiao-hong,WANG Zhen,SHENG Qi. Case Report and Review of Literature of Child Subcutaneous Panniculitis-Like T-Cell Lymphoma[J]. Journal of Applied Clinical Pediatrics, 2008, 23(24) |
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| Authors: | YUAN Xiao-juo ZHANG Qin YAO Xiao-hong WANG Zhen SHENG Qi |
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| Affiliation: | YUAN Xiao-jun1,ZHANG Qin1,YAO Xiao-hong2,WANG Zhen1,SHENG Qi1(1.Department of Pediatrics,2.Department of Pathology,Xinhua Hospital Affiliated to Medical College,Shanghai Jiaotong University,Shanghai 200092,China) |
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| Abstract: | 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 SPTCL with over 2 years protracted course of fever and multiple skin lesions.The evolvement of clinical presentation,the misdiagnosed experience,the histopathological features,the immunohistochemical resul... |
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| Keywords: | lymphoma skin T cell panniculitis child |
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