Wang Ting-ting, Wang Lin, Tang Zhi-rong, Cheng Ji-rong, Li Wei, Li Feng-yuan, Wang Wei-ya, Li Gan-di
Department of Dermatovenereology, West China Hospital, Sichuan University, Chengdu 610041, China.
Zhonghua Bing Li Xue Za Zhi. 2009 Nov;38(11):749-53.
To study the clinicopathologic features, immunophenotype and prognosis of primary cutaneous anaplastic large cell lymphoma (C-ALCL).
Eight cases of C-ALCL were enrolled into the study. The clinicopathologic features, immunohistochemical findings and results of in-situ hybridization for EBER 1/2 were analyzed.
Three of the 8 patients were males and 5 were females. The median age was 49.5 years. C-ALCL often presented with solitary skin nodule, without systemic symptoms. Histologically, the lymphoma cells infiltrated the dermis and subcutis in a sheet-like pattern. They were of large size and showed conspicuous nuclear atypia. Immunohistochemical study showed that more than 75% of the lymphoma cells were positive for CD30. All cases expressed one to three T cell markers (CD3, CD5 or CD45RO) and cytotoxic granule-associated antigens (TIA-1, granzyme B or perforin). The staining for leukocyte common antigen was positive in all cases, while the expression of CD5, CD8, ALK-1 and epithelial membrane antigen was noted in 5, 1, 1 and 3 cases, respectively. The staining for CD15, CD20, CK and HMB45 was negative. In-situ hybridization for EBER 1/2 was also negative in all the cases studied. Follow-up information was available in 6 patients. Five of them were still alive and 1 died of unclear cause.
C-ALCL has distinctive clinicopathologic and immunophenotypic features. It is not Epstein-Barr virus-related and often carries a favorable prognosis.
研究原发性皮肤间变性大细胞淋巴瘤（C-ALCL）的临床病理特征、免疫表型及预后。
纳入8例C-ALCL患者进行研究。分析其临床病理特征、免疫组化结果及EBER 1/2原位杂交结果。
8例患者中男性3例，女性5例。中位年龄为49.5岁。C-ALCL常表现为孤立性皮肤结节，无全身症状。组织学上，淋巴瘤细胞呈片状浸润真皮和皮下组织。细胞体积大，核异型明显。免疫组化研究显示，超过75%的淋巴瘤细胞CD30阳性。所有病例均表达一至三种T细胞标志物（CD3、CD5或CD45RO）及细胞毒性颗粒相关抗原（TIA-1、颗粒酶B或穿孔素）。所有病例白细胞共同抗原染色均为阳性，而CD5、CD8、ALK-1及上皮膜抗原的表达分别见于5例、1例、1例及3例。CD15、CD20、CK及HMB45染色均为阴性。所有研究病例的EBER 1/2原位杂交也均为阴性。6例患者有随访信息。其中5例仍存活，1例死因不明。
C-ALCL具有独特的临床病理及免疫表型特征。它与EB病毒无关，预后通常良好。
