原发皮肤间变大细胞淋巴瘤临床诊治分析

Primary cutaneous anaplastic large cell lymphoma： clinical presentation, therapy and prognosis study of 10 cases

目的 探讨原发皮肤间变大细胞淋巴瘤（PCALCL）的临床表现、治疗及预后特点.方法 回顾性分析中国医学科学院肿瘤医院1999年1月至2009年1月收治的10例PCALCL患者的临床资料.结果 中位发病年龄48岁（22～69岁）,男8例,女2例.皮损单发患者7例,多发3例.初期皮损呈红色皮下结节,质地较硬,多固定,部分皮损可呈自限性或反复出现,增大后可出现表面溃疡,呈菜花状.皮损可位于头颈部（5例）、躯干（3例）或全身各处（2例）.有2例淋巴结受侵和1例骨受侵,骨受侵者有间变大细胞淋巴瘤激酶（ALK）阳性和细胞增殖指数偏高（ki-67＞80%）.初始治疗：7例单发病灶患者采用手术切除后进行放疗、化疗或化放疗,除1例复发外,6例均无病生存;3例多发病灶患者采用化疗联合放疗或生物治疗,2例复发,1例无病生存.中位随访44个月（9～95个月）时,患者的无进展生存率为89%,总生存率为100%.结论 PCALCL多见于中年男性,不易侵犯内脏器官和淋巴结.病变局限性者,经治疗预后好;病变广泛者即使采用手术、放疗联合化疗,多数仍不能治愈.

Objective To explore the clinical presentation, therapy and prognosis study of primary cutaneous anaplastic large cell lymphoma （PCALCL）. Methods We reviewed and analyzed ten cases of PCALCL receiving treatment at our hospital from January 1999 to January 2009. Results There were 8 males and 2 females with a median age of 48 years old（ range： 22 -69）. There were single subcutaneous nodule （ n = 7 ） and multiplenodules （ n = 3 ）. And the lesions could be found on head and neck （ n = 5 ）,trunk （ n = 3 ） and all over body （ n = 2 ）. The lesions appeared red, solid and stable subcutaneous nodules.Partial lesions had a spontaneous regression and new nodules appeared at the same or different sites. Two patients had lymphadenopathy and one had bone involvement with anaplastic lymphoma kinase （ALK）positive and high cell proliferation ratio index （ ki-67 〉 80% ）. Seven cases with single lesion received surgical excision plus radiotherapy, chemotherapy or radiochemotherapy, one case recurred, six cases survived without disease. Three cases with multiple lesions received systemic chemotherapy mainly in combination with radiotherapy or biotherapy, two cases recurred and one case survived without disease. The median follow-up was 44 months （ range： 9 - 95 ）, progression free survival 89% and overall survival 100%. Conclusion PCALCL is found more commonly in males. Visceral and lymph node involvement are rare. The patients with single lesion have a longer disease-free survival than those with multiple lesions after surgical excision in combination with chemotherapy or radiotherapy. Multiple lesions can not be cured.