【摘要】 目的 探讨伴有惰性T淋巴母细胞增生(iT-LBP)性疾病的临床病理学特征、诊断及鉴别诊断。方法 分析2例伴有iT-LBP的Castleman病的临床特征、免疫表型及基因重排检测结果，并复习总结伴有iT-LBP的相关文献。结果 镜下观察可见典型的Castleman病改变，滤泡间区可见多量淋巴母细胞样细胞，免疫表型表达TdT及T细胞标记物，细胞增生活跃；T细胞受体重排检测未发现克隆性重排，诊断为伴有惰性T淋巴母细胞增生的Castleman病。总结文献报道及本组iT-LBP共25例，发现iT-LBP最常见于Castleman病和/或滤泡树突状细胞肉瘤，均伴有TdT及T细胞标记物阳性表达，且无T细胞克隆性重排。随访发现无一例死于iT-LBP。结论 iT-LBP常伴发于其他疾病，临床呈惰性过程，诊断时应进行仔细的形态学观察、免疫表型的检查、TCR检查以及密切的临床随访，然后作出准确的诊断，避免误诊引起不必要的治疗。更多还原

【Abstract】 Objective To investigate the clinicopathological characteristics, diagnosis and differential diagnosis of Castleman disease with indolent T-lymphoblastic proliferation(iT-LBP). Methods The clinical features, immunophenotype and gene rearrangement test results were analyzed in two cases of Castlemans disease with iT-LBP, and relevant literatures were reviewed and summarized. Results The presence of typical Castleman’s disease changes was observed under microscope. Multiple lymphocytoblast-like cells were observed in the interfollicular area. Immunophenotyping showed that interfollicular lymphocytoblast-like cells expressed TdT and T cell markers, and cell proliferation was active. T cell receptor rearrangement assay did not detect clonal rearrangement. Diagnosis was Castlemans disease with iT-LBP. A total of 25 cases of iT-LBP(included our case) were summarized; it was most commonly found in Castleman’s disease and/or follicular dendritic cell sarcoma. All were accompanied by positive expression of TdT and T cell markers, and there was no clonal rearrangement of T cells. Most patients were followed up and no one died from iT-LBP. Conclusion iT-LBP is often associated with other diseases and presents indolent when diagnosis; careful morphologic observation, immunophenotype and TCR gene rearrangement analysis along with close clinical follow-up should be carried out, and then accurate diagnosis should be made to avoid unnecessary treatment caused by misdiagnosis.更多还原