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目的探讨原发性皮肤间变性大细胞淋巴瘤(ALCL)的临床病理形态特征、免疫组化及预后特点。方法对13例原发性皮肤ALCL进行形态学观察,免疫组化标记及随访,并结合相关文献进行讨论。结果 13例原发性皮肤ALCL中,男女之比为1.6∶1,平均年龄47岁。临床表现为皮肤斑块、结节、肿块;组织学形态多样;免疫组化示CD30均(+),大多数CD3和/或CD43(+),部分EMA、GranB和perforin(+),ALK、CKpan、CD20、CD79a、HMB45、CD68、CD15和CD117(-)。13例均经外科手术切除局部病变,再辅助以化疗和∕或放疗;9例随访时间5～55个月,1例死亡。结论原发性皮肤ALCL是低度恶性淋巴瘤,预后相对较好。诊断依赖于组织病理学及免疫组化标记。本病应与弥漫性大B细胞性淋巴瘤、黑色素瘤、低分化腺癌等鉴别。
Objective To investigate the clinicopathological, immunohistochemical and prognostic features of primary cutaneous anaplastic large cell lymphoma (ALCL). Methods Thirteen cases of primary cutaneous ALCL were observed morphologically, immunohistochemically marked and followed up, and the related literatures were discussed. Results Thirteen patients with primary cutaneous ALCL had a ratio of men to women of 1.6:1 with an average age of 47 years. The clinical manifestations were skin patches, nodules and lumps. The histological morphology was diverse. Immunohistochemistry showed CD30 (+), most CD3 and / or CD43 (+), some EMA, GranB and perforin (+), ALK, CKpan, CD20, CD79a, HMB45, CD68, CD15 and CD117 (-). Thirteen patients underwent surgical resection of local lesion followed by chemotherapy and / or radiotherapy. Nine patients were followed up for 5 to 55 months and 1 died. Conclusion Primary skin ALCL is a low-grade lymphoma with a relatively good prognosis. Diagnosis depends on histopathology and immunohistochemical markers. The disease should be diffuse large B cell lymphoma, melanoma, poorly differentiated adenocarcinoma and other identification.