摘要目的 探讨原发皮肤间变性大细胞淋巴瘤(CALCL)多样的临床病理特征、免疫表型,并行预后相关性分析.方法 收集CALCL 44例,总结临床病理资料、治疗情况和随访资料.常规HE染色和免疫组织化学EnVision染色行组织病理形态学观察.结果 44例CALCL患者临床皮损以结节、肿块和(或)斑块为主,可伴溃疡形成.四肢最常受累.39例获得随访,生存率为87.2%(34/39).46.2%(18/39)患者有疾病复发,统计学分析显示年龄＞50岁与累犯≥2个解剖部位的病例组复发率显著升高(P＜0.05).44例CALCL中,31例为经典型,6例为小细胞变型,另有7例为富于中性粒细胞/嗜酸性粒细胞变型.CD30、CD45、CD45RO、CD43、CD3、细胞毒性蛋白和上皮细胞膜抗原的阳性率分别为100%(44/44)、91.2%(31/34)、82.6%(19/23)、94.7%(18/19)、70.0%(28/40)、73.3%(22/30)和31.8%(7/22).CD4~+/CD8~+、CD4~-/CD8~+和CD4~-/CD8~-免疫表型的比例分别为58.3%(21/36)、22.2%(8/36)和19.4%(7/36).仅1例(3.7%)同时表达CD56.结论 CALCL是一类低度恶性皮肤T细胞淋巴瘤,组织学形态多变,认识CALCL特殊类型有助于避免与其他类型淋巴瘤和炎症性病变相混淆.年龄＞50岁和累犯≥2个解剖部位CALCL患者与高复发率相关.

abstractsObjective To study the cilnicopathologic features,immunophenotype and prognosis of primary cutaneous anaplaatic large cell lymphoma(CALCL).Methods Histopathologic evaluation and immunohistochemical study by Envision method were carried out in 44 archival cages of CALCL The clinical information and foilow-up data were analyzed.Results The patients presented with skin nodules,masses or plaques,sometimes associated with ulceration.The commonest sites of involvement were the extremities.Follow-up data were available in 39 patients.The overall snrvival rate was 87.2%(34/39).Disease relapses were detected in 46.2%(18/39)of the patients.Statistical analysis indicated that patients older than 50 years of age or with no less than two involved anatomic sites were more likely to have disease relapses (P＜0.05).Histologically,31 cases were classified as common variant,6 cases as small cell variant and 7 cases as neutrophil/eosinophil-rich variant.Immunohistochemical study showed that the rates of expression of CD30,CD45,CD45RO,CD43,CD3,cytotoxic protein and epithelial membrane antigen were 100%(44/44),91.2%(31/34),82.6%(19/23),94.7%(18/19),70.0%(28/40),73.3%(22/30)and 31.8%(7/22),respectively.The CD4~+/CD8~-,CD4~-/CD8~+ and CD4~-/CD8~- immunophenotypes were found in 58.3%(21/36),22.2%(8/36)and 19.4%(7/36)of the CALCL cases,respectively.Only one case(3.7%)expressed CD56.Conclusions CALCL is a form of low-grade primary cutsneous T-cell lymphoma with a wide spectrum of clinicopathologic pattern.Special variants of CALCL should not be confused with other types of cutaneous lymphomas and inflammatory lesions.CALCL patients older than 50 years of age or with no less than two involved anatomic sites are more likely to have disease relapses.