皮下脂膜炎样T细胞淋巴瘤的病理分析及基因诊断

Clinicopathological analysis and genetic diagnosis of subcutaneous panniculitic T-cell lymphoma

目的 :探讨皮下脂膜炎样T细胞淋巴瘤 (SPTCL)的基因诊断方法及病理特点。方法 :在组织学诊断的基础上 ,辅以免疫组织化学 ,并利用PCR技术检测石蜡切片标本IgH及TCRβ基因重排。 结果 :将 3例原考虑为结节性非化脓性脂膜炎的病例确诊为皮下脂膜炎样T细胞淋巴瘤。患者均为青壮年 ,起病较急 ,表现为皮下结节伴红斑 ,镜下见异型淋巴细胞浸润皮下脂肪组织间质 ,呈脂膜炎样改变 ,可见核碎裂、局灶性脂肪坏死及泡沫状组织细胞。免疫表型 :LCA + ,UCHL 1+ ,Mac387- ,TCR β基因重排 + ,IgH基因重排 -。 结论 :皮下脂膜炎样T细胞淋巴瘤具有较特殊的病理组织学特征 。

Purpose To explore the clinicopathological features of subcutaneous panniculitic T cell lymphoma(SPTCL) and significances of genetic analysis in the diagnosis. Methods Histopathology, immunohistochemitry and detection of clonal gene rearrangement by PCR were used in 3 cases of subcutaneous panniculitic T cell lymphoma (SPTCL), which were originally diagnosed as relapsing nodular nonsuppurative panniculitis. Results Three misdiagnostic cases were correctly redefined as subcutaneous panniculitic T cell lymphoma, with immunophenotype of CD45+,CD45RO+, Mac387-,and clonal TCR β gene rearrangement. Conclusions Subcutaneous panniculitic T cell lymphoma has distinctive clinicopathological features. Genetic analysis is an effective method for the diagnosis of SPTCL.