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目的提高对发生于鼻咽部的母细胞性浆细胞样树突状细胞肿瘤(BPDCN)的诊断与鉴别诊断水平。方法对1例以鼻咽部肿物为主要特征的BPDCN的临床病理特征、免疫组化、分子检测以及治疗与预后情况进行分析,并复习文献资料。结果患者女性,33岁。以鼻咽部占位伴颈部淋巴结肿大就诊。鼻咽部活组织检查示肿瘤细胞由小~中等大小细胞组成,细胞质稀少,核形略不规则,染色质细腻,核仁不明显。骨髓穿刺示肿瘤由小~中等大小细胞组成,伴大量坏死。追问病史并详细体检发现左前臂皮下包块。免疫组化示肿瘤细胞CD4、CD56和CD123(+),CD3、CD20和CD79α(-);鼻咽部活检物原位分子检测示EBER(-),T系基因重排检测(-)。最后确诊为BPDCN,肿瘤累犯鼻咽部、皮肤及骨髓。结论以累犯鼻咽部为主要临床特征的BPDCN极易误诊为该部位最常见的NK/T细胞淋巴瘤,临床主要应与NK/T细胞淋巴瘤、侵袭性NK细胞白血病等相鉴别。
Objective To improve the diagnosis and differential diagnosis of nasal pharyngeal plasmacytoid dendritic cell tumor (BPDCN). Methods A case of BPDCN characterized by nasopharyngeal masses was analyzed for its clinicopathological features, immunohistochemistry, molecular detection, treatment and prognosis, and the review of the literature. Results Female patient, 33 years old. To nasopharyngeal space occupancy with cervical lymph nodes treatment. Nasopharyngeal biopsy showed tumor cells from small to medium size cells, sparse cytoplasm, nuclear slightly irregular, delicate chromatin, nucleoli are not obvious. Bone marrow puncture shows the tumor from small to medium size cells, with a large number of necrosis. Asked history and detailed physical examination found that the left forearm subcutaneous mass. Immunohistochemistry showed that CD4, CD56 and CD123 (+), CD3, CD20 and CD79α (-) were detected by immunohistochemistry. In situ molecular detection of nasopharyngeal biopsy showed EBER (-), T gene rearrangement assay. The final diagnosis of BPDCN, tumor recidivism nasopharynx, skin and bone marrow. Conclusions BPDCN with recidivism of nasopharynx as the main clinical features can be easily misdiagnosed as the most common NK / T cell lymphoma in this area. The main clinical manifestations should be differentiated from NK / T cell lymphoma and invasive NK cell leukemia.