原发性皮肤间变性大细胞淋巴瘤临床病理分析  

Primary cutaneous anaplastic large cell lymphoma： a clinicopathologic analysis of 8 cases

作　　者：王婷婷[1] 王琳[1] 唐治容 程继荣[4] 李薇[1] 李俸媛[2] 王威亚[2] 李甘地[2] 

机构地区：[1]四川大学华西医院皮肤科,成都610041 [2]四川大学华西医院病理科,成都610041 [3]四川省南充市中心医院病理科 [4]四川省达州市中心医院病理科

出　　处：《中华病理学杂志》2009年第11期749-753,共5页Chinese Journal of Pathology

基　　金：基金项目：2005年四川大学华西医院留学回国人员科研启动基金（136050122）

摘　　要：目的探讨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。方法分析8例C—ALCL的临床病理资料，复习HE切片，进行T淋巴细胞、B淋巴细胞、活化淋巴细胞和细胞毒性等16种标记的免疫组织化学染色，原位杂交检测EB病毒。结果8例中男3例，女5例，中位年龄49．5岁。临床上以皮肤无症状的单个红色结节、肿块为主要表现，组织学上肿瘤细胞在真皮与皮下脂肪内大片状、弥漫性浸润。瘤细胞以大细胞为主，异形性明显。8例C—ALCL的瘤细胞CD30阳性细胞数均大于75％。瘤细胞均表达1～3个T细胞标记（CD3、CD5或CD45RO）及1～3个细胞毒性标记[T细胞内抗原（TIA）-1、颗粒酶B或穿孔素]。表达白细胞共同抗原（LCA）为8例、CD4为5例、CD8为1例、间变性淋巴瘤激酶（ALK）-1为1例、上皮细胞膜抗原（EMA）为3例，均不表达CD15、CD20、CK和HMB45。EBER1／2原位杂交均为阴性。获随访的6例中5例存活，1例死亡（死因不详）。结论C—ALCL有独特的临床病理表现和免疫表型，预后较好。EB病毒与C—ALCL可能无明确的相关性。Objective To study the clinicopathologic features, immunophenotype and prognosis of primary cutaneous anaplastie large cell lymphoma （ C-ALCL）. Methods Eight cases of C-ALCL were enrolled into the study. The elinicopathologic features, immunohistochemieal findings and results of in-situ hybridization for EBER 1/2 were analyzed. Results 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 subeutis 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 CDS, 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. Conclusions C-ALCL has distinctive clinicopathologic and immunophenotypic features. It is not Epstein-Barr virus-related and often carries a favorable prognosis.

关 键 词：淋巴瘤 大细胞 Ki-1 淋巴瘤 大细胞 免疫组织化学 皮肤 

分 类 号：R686[医药卫生—骨科学]