原发性皮肤间变大细胞淋巴瘤临床特征及预后分析

目的:探讨原发性皮肤间变大细胞淋巴瘤（PC-ALCL）患者的临床特征及预后。方法:回顾性分析2008年08月至2019年09月我院血液科收治的7例PC-ALCL患者临床特征及预后。结果:7例患者，男性3例，女性4例，中位年龄为52岁（15~73岁）。孤立性病灶1例，区域性病灶2例，广泛性病灶4例。病理亚型6例为ALK阴性，1例为ALK阳性。与孤立性和区域性病灶患者不同，广泛性病灶患者具有更高的铁蛋白、乳酸脱氢酶、β2-MG。7例患者均行全身化疗，主要采用CHOP样方案，而接受手术、放疗各1例。7例患者的中位随访时间为67个月（0.6~152个月），5年OS为85.7%。1例ALK阳性孤立性病灶患者，经手术及化疗，原发病持续CR，OS为152个月。2例区域性病灶患者，原发病分别持续PR和CR。4例广泛性病灶患者5年OS为50%，2例死亡患者中，1例接受多次化疗及自体外周血造血干细胞移植，OS为 67个月；另 1例诊断时存在睾丸、附睾浸润，OS仅为0.6个月。结论:PC-ALCL是相对罕见的ALCL亚型，中年高发，病理类型以ALK阴性为主，孤立性和区域性PC-ALCL预后较好，广泛性PC-ALCL具有更高的铁蛋白、乳酸脱氢酶、β2-MG，且预后更差；ALK阳性PC-ALCL相对少见，但预后良好。

Analysis of clinical characteristics and prognosis in patients with primary cutaneous anaplastic large cell lymphoma

Objective:To investigate the clinical characteristics and prognosis in patients with primary cutaneous anaplastic large cell lymphoma(PC-ALCL).Methods:We conducted a retrospective review of seven patients with PC-ALCL diagnosed and treated in the department of hematology of our hospital from August 2008 to September 2019.Results:We identified 7 eligible patients,3 males and 4 females,with a median age of 52 years(15~73 years).The biopsy results were as follows:Isolated foci in one was ALK-positive,localized sites in two and multifocal in four were ALK-negative.Compared to patients with isolated and localized lesions,cases with multifocal lesions had increased ferritin,lactate dehydrogenase(LDH),and β2-MG.All patients received systemic chemotherapy,mainly CHOP-like regimens.In addition,the patient underwent surgery and radiotherapy was one respectively.The median follow-up was 67 months(0.6~152 months),and the 5-year OS rate was 85.7%.One patient with ALK-positive solitary lesion was in continuous CR with OS 152 months.Two patients with localized lesions sustained PR and CR respectively.The 5-year OS of the four patients with extensive lesions was 50%.One of the deaths received multiple chemotherapies and autologous hematopoietic stem cell transplantation,and OS was 67 months.The other case had testis and epididymis infiltration at diagnosis with OS only 0.6 months.Conclusion:PC-ALCL is a rare subtype of ALCL with high incidence in middle age.The prognosis of isolated and localized PC-ALCL is good,while the multifocal patient with increased ferritin,LDH,and β2-MG is poor.The pathological type is mainly ALK-negative,and ALK-positive is relatively rare,but the prognosis is better than ALK-negative.