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目的:报道1例皮下脂膜炎样T细胞淋巴瘤,探讨此类少见的皮肤T细胞淋巴瘤的临床和病理组织学特征以及治疗预后。方法:分析1例皮下脂膜炎样T细胞淋巴瘤患者的临床资料并通过复习文献,总结该病的临床病理、免疫表型、治疗及预后特点。结果:患者临床表现为发热、皮疹,皮肤活检病理示,真皮及皮下组织内大量核深染、形状不规则单个核细胞。免疫组化示,CD45RO弥漫阳性,CD3阳性,CD4阴性,CD8阳性,CD30部分阳性,TIA-1阳性,CD20阴性。CHOP方案化疗后一度好转,但很快复发,死于严重感染和多脏器功能衰竭。结论:皮下脂膜炎样T细胞淋巴瘤是一种少见的皮肤T细胞淋巴瘤,预后差,免疫表型和T细胞受体基因重排检测在该病的诊断、治疗及预后等方面起着关键的作用。
OBJECTIVE: To report one case of subcutaneous panniculitis-like T-cell lymphoma and to explore the clinical and histopathological features and prognosis of such rare cutaneous T-cell lymphoma. Methods: The clinical data of 1 patient with subcutaneous panniculitis-like T-cell lymphoma were analyzed and the clinical pathology, immunophenotype, treatment and prognosis were summarized through reviewing the literature. Results: The clinical manifestations of the patients were fever, skin rash, pathological examination of skin biopsy, a large number of nuclear stained and irregularly shaped mononuclear cells in the dermis and subcutaneous tissue. Immunohistochemistry showed that CD45RO diffuse positive, CD3 positive, CD4 negative, CD8 positive, CD30 partially positive, TIA-1 positive, CD20 negative. CHOP regimen once improved after chemotherapy, but relapse soon, died of severe infection and multiple organ failure. Conclusion: Subcutaneous panniculitis-like T-cell lymphoma is a rare cutaneous T-cell lymphoma. The prognosis is poor. The detection of immunophenotype and T cell receptor gene rearrangement play an important role in the diagnosis, treatment and prognosis of the disease The key role.