皮下脂膜炎样T细胞淋巴瘤

Subcutaneous panniculitis-like T-cell lymphoma

目的 :探讨皮下脂膜炎样T细胞淋巴瘤的临床和病理特征。方法 :对 8例皮下脂膜炎样T细胞淋巴瘤进行组织细胞学和免疫组化染色观察 ,结合文献复习总结其临床和病理特征。结果 :临床上首发症状均表现为皮下结节 ,单发或多发 ,继而向上侵及表皮 ,向下累及筋膜。半数病例长期稳定 ,无系统症状 ,治疗效果好 ,长期存活。另半数病例出现高烧 ,累及骨髓、淋巴结 ,肝脾肿大 ,血小板或全血象减少 ,少数伴有致死性嗜血细胞综合征 ,病程进展迅速 ,极少存活 3年者。病理上瘤细胞由异型的大、中、小淋巴细胞以不同比例围绕脂肪细胞呈花环状排列 ,融合成小片状或灶状呈网格状分布。瘤细胞核分裂象活跃 ,有明显的核碎裂小体构成的核碎裂坏死。周围区组织细胞反应性增生活跃伴有吞噬红细胞现象 ,并见多核巨细胞和肉芽肿样反应。瘤细胞浸润在脂肪小叶内 ,脂肪小叶间隔则不累及。肿瘤表达T细胞免疫表型。结论 :本瘤是累及皮下脂肪组织的 。

Purpose To inquire into the clinical and pathological features of subcutaneous panniculitis like T cell lymphoma. Methods The histopathology and immunophenotypes of subcutaneous panniculitis like T cell lymphoma in 8 cases were observed. Results Clinically one or more subcutaneous nodules were first found, then the lesion infiltrated up to the epiderm or down to the fascia. Half of the cases were stable for long time without systematic symptoms. The others got high fever with bone marrow, lymphonode involvement, had tumescent liver, and spleen, and decreasing of the counts of thrombocytes or whole blood cells. A few were accompanied with lethal hematophage syndrome. Some rapidly advanced and died less than 3 years. Pathologically, atypical large, midium size and small lymphocytes with variable ratio rounded the lipocytes like rosettes and were confluent to small patchs or foci or formed a reticular pattern. There were many karyokineses and karyorrhexis made of distinct karyorrhexis bodies. The histocytes reactively proliferated accompanied with phagoerythrocytic phenomena, and polynuclear giant cells and granulomatoid reaction were found. The tumor cells infiltrated in the lipolubules. They expressed T cell immunophenotype. Conclusions This tumor is a special type of skin lymphoma involving subcutaneous fat tissue and special clinicophathological features.