间变性大细胞淋巴瘤的形态学及免疫表型与诊断预后的相关性

Anaplastic Large Cell Lymphoma:Correlation of Clinicopathologic Features with Diagnosis and Prognosis

目的:研究间变性大细胞淋巴瘤(ALCL)的临床病理特点和免疫表型,提高该疾病的早期诊断,并比较ALK+与ALK-在临床表现、治疗和预后的不同点。方法:对原诊断为ALCL的20例病例重新行组织学观察和免疫组织化学染色(ABC法)并复习相关文献。结果:20例中15例属于T/Null细胞表型,结合免疫组化诊断为ALCL,15例ALCL均强烈表达CD30;ALK阳性者9例,ALK的表达与患者性别,免疫分型、组织分型、临床分期无相关性;而与患者年龄及化疗敏感性具有相关性(P<0.05)。其余5例为B细胞表型(CD20强阳性),结合新的淋巴瘤分类标准,被诊断为弥漫性大B细胞淋巴瘤(DLBCL)。结论:间变性大细胞淋巴瘤中具有标志性的大细胞出现CD30、ALK和EMA阳性时,对ALCL的诊断具有重要价值;ALK+ALCL与ALK-ALCL在遗传特征,临床表现及治疗预后的不同,使其作为一种新的病理类型被提出具有合理性。

Objective： To investigate the clinicopathological and immunohistochemical characteristics of anaplastic large cell lymphoma （ALCL） and to compare the differences in clinical manifestation, treatment and prognosis between ALK-positive cases and ALK-negative cases. Methods Histological and immunohistochemical staining methods （ABC method） were used to analyze 20 previously diagnosed cases of ALCL, and the correlated clinical data were reviewed. Results： Fifteen of the 20 cases were T/Null cell type （8 T and 7 Null） and were di- agnosed as ALCL with immunohistochemistry. CD30 was strongly expressed in the 15 cases of ALCL. There were 9 ALK-positive cases. No correlation was found between ALK expression and sex, immunohistochemical type, histological type, or clinical stage. However, a significant correlation was found between ALK expression and age and sensitivity to chemotherapy （P〈0.05）. The remaining 5 cases were B cell type, and they were true DLBCL. Conclusion： Our study highlights the importance of histopathological features and the significance of CD30 and ALK in the diagnosis of ALCL. The differences between ALK-positive ALCL and ALK-negative ALCL suggest specific studies on these genes, including differences in clinical manifestation and remission rates after chemotherapy, should be performed.