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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 clinical and pathological features of anaplastic large cell lymphoma (ALCL). Methods: The clinical data of 12 patients with systemic ALCL were retrospectively analyzed. The microscopic morphology and immunophenotypic features of the pathological tissues were analyzed. The related fusion genes were detected by fluorescence in situ hybridization and the related literatures were reviewed. Results: There were 6 males and 6 females in each of 12 ALCL patients, ranging in age from 22 to 81 years. 9 cases were accompanied by repeated fever, weight loss, night sweats, anemia and other symptoms of B; 5 cases of ALCL occurred in lymph nodes, 3 cases involving both lymph nodes. Microscopically, most of the ALCL lesions were pleomorphic cells. All of them showed characteristic renal cell nuclei. Some tumors showed adherent growth. One case showed small cell variant and two cases showed sarcomatoid differentiation. Immunohistochemistry showed that ALK was positive in 4 cases and negative in 8 cases. CD30 was expressed in all 12 cases, CD2 was positive in 7 cases, CD3 was positive in 5 cases, CD5 was positive in 3 cases, CD7 was positive in 2 cases, and CD4 / CD8 was also found in 2 cases Positive, 5 cases of different degrees of expression of cytotoxic markers. Four cases of immunohistochemical ALK positive by fluorescence in situ hybridization were detected by isolated signal. Conclusion: The symptoms of B in ALCL patients are common and easily lead to the extranodal tissues. The tumor tissues are diverse in morphology under light microscopy, and some T cell-associated antigens are often lost. Combined application of T cell markers is of great significance in diagnosis and differential diagnosis.