CAJ | 학술논문

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、CIM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.EBER 1/2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.
목적 탐토원발성피부간변성대세포림파류(C-ALCL)적림상병리특정、면역표형급예후.방법 분석8례C-ALCL적림상병리자료,복습HE절편,진행T림파세포、B림파세포、활화림파세포화세포독성등16충표기적면역조직화학염색,원위잡교검측EB병독.결과 8례중남3례,녀5례,중위년령49.5세.림상상이피부무증상적단개홍색결절、종괴위주요표현,조직학상종류세포재진피여피하지방내대편상、미만성침윤.류세포이대세포위주,이형성명현.8례C-ALCL적류세포CD30양성세포수균대우75%.류세포균표체1～3개T세포표기(CD3、CD5혹CD45RO)급1～3개세포독성표기[T세포내항원(TIA)-1、과립매B혹천공소].표체백세포공동항원(LCA)위8례、CIM위5례、CD8위1례、간변성림파류격매(ALK)-1위1례、상피세포막항원(EMA)위3례,균불표체CD15、CD20、CK화HMB45.EBER 1/2원위잡교균위음성.획수방적6례중5례존활,1례사망(사인불상).결론 C-ALCL유독특적림상병리표현화면역표형,예후교호.EB병독여C-ALCL가능무명학적상관성.
Objective To study the clinicopathoiogic features, immunophenotype and prognosis of primary cutaneous anaplastic large cell lymphoma (C-ALCL). Methods 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. 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 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. Conclusions C-ALCL has distinctive clinicopathologic and immunophenotypic features. It is not Epstein-Barr virus-related and often carries a favorable prognosis.