间变性大细胞淋巴瘤的p80蛋白的表达及其临床意义

The expression and clinical significance of p80 protein in anaplastic large cell lymphoma

目的 研究染色体易位t( 2 ;5 )所形成的嵌合基因NPM ALK的蛋白产物———p80蛋白在间变性大细胞淋巴瘤 (ALCL)中的表达及与其亚型和预后的关系。方法 对已进行临床、病理和免疫学分析并有随访资料的 19例ALCL用免疫组织化学ABC法标记p80蛋白。结果 p80蛋白在 19例中有 9例呈强阳性 ,组织学亚型为普通型和小细胞型 ,无 1例为霍奇金样型 (P <0 .0 5 )。p80蛋白阳性病例的免疫学表型为T细胞性 8例和Null细胞性 1例 ,无B细胞性和T、B细胞标记均表达的病例 (P<0 .0 5 )。p80蛋白阳性病例发病年龄小于阴性病例 (P <0 .0 5 ) ,且对化疗的反应及预后均较阴性者差 (P <0 .0 5 )。结论 ( 1)存在染色体易位t( 2 ;5 )所形成的嵌合基因NPM ALK蛋白 ;表达p80蛋白的ALCL可能为新的独立的淋巴瘤实体 ;( 2 )检测p80蛋白可用于ALCL的诊断。

Objective To investigate the relationship of expression of NPMALK cohesive gene protein with the subtypes and prognosis of anaplastic large cell lymphoma (ALCL). Methods Nineteen cases of ALCL with CD30 positivity were studied. Immunohistochemical staining was performed in paraffin sections by using polyclonal antibody antip80 and other monoclonal antibodies. The clinical data of these patients were analyzed and all patients were followedup. Results The neoplastic cells in 9 out of 19 ALCL expressed p80 protein. Histopathologically, all 9 cases with p80 expression were common type and small cell type. None of these 9 cases were Hodgkin like type (P<0.05). Immunologically, all the 9 positive cases were T and null cell phenotype and none of them was B cell or other phenotype (P<0.05).Clinically, the patients with p80 expression were younger than those without p80 expression (P<0.05). The prognosis of the former was worse than in the later (P<0.05). Conclusions p80 positive ALCL could be a new clinicopathological entity. The polyclonal antibody antip80 might be an useful marker for the differential diagnosis and prognosis estimation of ALCL.