儿童皮下脂膜炎样T细胞淋巴瘤临床病理观察

目的研究皮下脂膜炎样T细胞淋巴瘤的临床病理特征、免疫表型、基因重排和预后。方法按照2005年WHO—EORTC分类中明确限定的SPTCL的诊断标准，分析我院收治的1例SPTCL患者的临床表现、病理组织学特点、免疫组织化学结果、T细胞受体（TCR）基因克隆性重排以及预后情况，并对相关文献进行复习。结果组织学上在皮下异型淋巴样细胞围绕单个脂肪细胞形成特征性的花边样结构。免疫组化肿瘤细胞表达：CD3、CD5、CD8、CD45RO、GranzymeB、TLA-1，不表述CD20、CD30、CD79a、CD56、PcK，TCR基因重排示：TCR3t基因重排JVI（-），JVⅡ（-）；TCRβ基因重排JD1（＋），JD2（-）支持为dBT细胞起源的淋巴瘤，患儿随访8个月内完全缓解。结论SPTCL是一种罕见的起源于α/β型T细胞的细胞毒性皮肤淋巴瘤，该肿瘤为惰性T细胞淋巴瘤，其病程迁延反复，早期治疗预后良好，免疫组化和基因重排对SPTCL的准确诊断是必不可少的。

Clinicopathologic Features of Subcutaneous Panniculitis-like T-cell Lymphoma in Children

Objective To study the elinicopathological features,the immunophenotyping,the gene rearrangement and the prognosis of sub- cutaneous panniculitis - like T - cell lymphoma （SPTCL）. Methods Based on the diagnosing criteria for SPTCL in WHO - EORTC Classi- fication made in 2005, an analysis was made to the clinical manifestation, the histopathlogical features, the results of immunohistocheical study, TCR clonal gene rearrangement and the prognosis of 1 case with SPTCL; the related literature was reviewed. Results The histological study revealed that the subcutaneous atypical lymphoid cells surrounded each single fat cell in a specific lace - like structure; immunohistochemical study revealed a positive expression of the tumor cells CD3, CDS, CD8, CD45RO, GranzymeB ,TIA - 1 and a negative expression of CD20, CD30, CIY79a, CD56, PCK; TCR gene rearrangement indicated JV I （ - ）, JV Ⅱ （ - ） of TCRT; TCRβgene rearrangement with JD1 （ ＋ ） and JI）2 （ - ） implied that the lymphoma was derived from β/β T - cell ; the follow - up covering 8 months showed a total relief in the patient. Conclusions SPTCL is rare and is derived fromγ/β T - cell; as an inertia T - cell lymphoma,it has a protracted clinical course, early treatment may lead to a better prognosis; immtmohistochemical study and gene rearrangement are critical for an accurate diagnosis of SPTCL.