皮下脂膜炎样T细胞淋巴瘤2例报告并文献复习

Subcutaneous panniculitis-like T-cell lymphoma: report of 2 cases and literature review

目的 探讨皮下脂膜炎样T细胞淋巴瘤 (SPTCL)的临床和病理特征。方法 报道 2例发生在四肢的多发性病灶的病例 ,并结合文献复习对其临床病理组织学特点、免疫组化、诊断、治疗及预后进行分析。结果 SPTCL主要表现为皮下结节 ,单发或多发。全身表现多种多样 ,可伴致死性噬血细胞综合征 ,临床进展迅速 ,于发病后 9个月及 2个月内死亡。组织病变均呈脂膜炎样改变 ,肿瘤细胞弥漫浸润皮下脂肪组织 ,可环绕脂肪空泡分布形成花边样结构。肿瘤表达T细胞免疫表型。结论 SPTCL是一种以累及皮下脂肪组织为主的特殊类型的淋巴瘤 ,预后差。

Objective To inquire into the clinical and pathological features of SPTCL. Methods Clinicopathological changes, immunophenotyping features, diagnosis, treatment and prognosis were studied in 2 cases of SPTCL. Results The main clinical manifestations of SPTCL was the single or multiple subcutaneous nodules. Systemic symptoms were variable. Patients may accompany a lethal hemophagocytic syndrome, which increasing rapidly, one died in 9 months, another died in 2 months. There were infiltration of atypical lymphocytes surrounding an adipose cell, in pannicultitis-like pattern, in subcutaneous tissue. The tumor cells infiltrated the lipolubuls. They expressed T-cell immunophenotype. Conclusion SPTCL is a specific type of lymphoma involving primarliy in subcutaneous fatty tissues, prognosis was poor.