间变性大细胞淋巴瘤临床病理特征分析

Anaplastic large cell lymphoma:a clinicopathological study and review of literature

目的 :探讨间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的临床和病理特点。方法:回顾12例系统性ALCL患者的临床资料,分析其病理组织的镜下形态、免疫表型特征,采用荧光原位杂交检测相关融合基因,并复习相关文献。结果:12例ALCL患者中男女各6例,年龄为22~81岁。9例伴有反复发热、体重减轻、盗汗、贫血等B症状;5例的ALCL发生于淋巴结结外,3例同时累及淋巴结。镜下,ALCL病灶以多形性细胞为主,均可见特征性肾形细胞核,部分肿瘤呈黏附性生长,1例为小细胞变异型,2例可见肉瘤样分化。免疫组织化学检测显示,4例患者ALK阳性,8例阴性;12例均有CD30表达,7例CD2阳性,5例CD3阳性,3例CD5阳性,2例CD7阳性,2例CD4/CD8标志均阳性,5例不同程度表达细胞毒标志。4例免疫组化ALK阳性者经荧光原位杂交均检测观察到分离信号。结论:ALCL患者B症状多见,易累及结外组织,肿瘤组织在光镜下形态多样,常失表达部分T细胞相关抗原,联合应用T细胞标志对其诊断及鉴别诊断具有重要意义。

Objective： To investigate the clinicopathological feature of anaplastic large cell lymphoma （ALCL）. Methods： A total of 12 cases of ALCL were enrolled and the clinicohistopathological characteristics, results of im- munophenotyping, fluorescence in situ hybridization （FISH） were analyzed. The relevant literatures were reviewed. Results： Patients were 6 males, 6 females, aged from 22 to 81 years, with an average of 47 years. B symptoms occurred in 9 cases. Extranodal lesions or also with lymph node lesions simultaneously were involved in 5 cases. Microscopically, ALCL was composed of pleomorphic large tumor cells predominantly, horseshoe-shaped nuclei cells were found in all cases, and adhesive growth pattern was seen in some cases. Small cell variant was seen in 1 case, and sarcomatoid features in 2 cases; 4 cases were positive for ALK, while 8 were negative for ALK. Positive staining for CD30 was seen in all the 12 cases, while positive for CD2 in 7 cases, CD3 in 5 cases, CD5 in 3 cases, CD7 in 2 cases, both positive for CD4/CD8 in 2 cases. In addition, cytotoxicity was found in various degree in 5 cases. Separating signal was found by FISH in all the 4 cases which were positive for ALK by immunohistochemical staining. Conclusions： ALCL patients usually present with B symptoms and tend to be involved in extranodal sites. Microscopic pathology reveals pleomorphic feature and often lacking some T lymphocyte-associated antigens. Combined detection of T cell markers is important for diagnosis and differential diagnosis.