中文摘要：

目的：探讨原发皮肤间变大细胞淋巴瘤（primary cutaneous anaplastic large cell lymphoma，PCALCL）的临床特征及治疗方法。方法：回顾性分析天津医科大学肿瘤医院2009年1月至2012年12月收治的6例PCALCL患者的临床资料。结果：中位发病年龄为54（38～60）岁，男女各3例；单发皮损3例，多发皮损3例。患者初期出现红斑或红色片状皮下结节样皮损，结节多高于皮面，界清、质硬，多不伴疼痛，部分患者伴皮损处瘙痒，结节逐渐增大，多发者相邻皮损可出现融合，部分皮损可表现为自限性或反复出现，搔抓后伴脱屑，随着病情发展，皮损颜色由红变暗，皮损表面可破渍。仅1例出现皮肤外浅表淋巴结侵犯。3例单发皮损患者完全手术切除后行辅助化疗和（或）放疗，均无病生存。3例多发皮损患者以化疗为主，2例出现复发，1例无病生存。中位随访时间24（11～35）个月，无进展生存率为66．7％，总生存率100％。结论：对于单发局限病灶，手术切除或放射治疗就可以获得满意的控制率。化疗可试用于控制多发病灶及有真皮外侵犯的患者。

英文摘要：

Objective： To explore the clinical presentation and therapy of primary cutaneous anaplastic large cell lymphoma （PCALCL）. Methods： We reviewed and analyzed six cases of PCALCL who were treated at our hospital from January 2009 to Decem- ber 2012. Results： The subjects were three males and three females with a median age of 54 years old （range： 38 years to 60 years）. Three patients had a single subcutaneous nodule and three had multiple nodules. The subcutaneous lesions appeared red, solid, and sta- ble nodules. Some patients developed pruritus around the lesions, or developed more nodules, which coalesced with other nodules. Some of the lesions exhibited partial spontaneous regression and new nodules appeared at the same site or at different sites. Finally, the lesions changed color from red to brown. The surfaces of some lesions developed ulcerations. Only 1 case had superficial lymph node metastasis. Three cases with single lesions received surgical excision plus radiotherapy, chemotherapy, or radiochemotherapy; all three cases survived and achieved complete remission. The three cases with multiple lesions mainly received systemic chemotherapy, with two of the cases recurring and one surviving with complete remission. The median follow-up was 24 months （range： 11 months to 35 months）, with a progression free survival of 66.7% and an overall survival of 100%. Conclusion： Surgical excision or radiotherapy provides satisfactory control in patients with single lesions. Chemotherapy may be prescribed to patients with multiple lesions or with extracutaneous metastasis.