伴惰性T淋巴母细胞增生的Castleman病2例并文献复习

Indolent T-lymphoblastic proliferation in Castleman disease:report of two cases and review of literature

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。

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 iTLBP.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.