母细胞性浆细胞样树突细胞肿瘤临床病理分析

Clinicopathological analysis of blast plasmacytoid dendritic cell neoplasm

目的探讨母细胞性浆细胞样树突细胞肿瘤的临床病理学特征。方法应用光镜及免疫组化法观察母细胞性浆细胞样树突细胞肿瘤的组织学特点及免疫表型,并复习相关文献。结果母细胞性浆细胞样树突细胞肿瘤好发于老年患者,多以皮肤病变为首发症状,形态学表现为肿瘤细胞呈弥漫、密集、单一性浸润真皮及皮下组织,细胞中等大小,细胞浆量少,核卵圆形或不规则,染色质细,核分裂易见。免疫组化肿瘤细胞阳性表达CD4、CD56、CD123;而CD2、CD3、CD5、CD8、GranB、TIA-1、CD20、CD79a、PAX-5、MPO阴性。结论 BPDCN是一种罕见的淋巴造血系统恶性肿瘤,临床侵袭性强,易复发,预后差,应注意与其他淋巴造血系统肿瘤相鉴别。

Objective To investigate the clinicopathological characteristics of blast plasmacytoid dendritic cell neo- plasm. Methods The histological features and immunophenotype of blast plasmacytoid dendritic cell neoplasm were ob- served by light microscopy and immunohistochemistry. The related literature was reviewed. Results Blast plasmacytoid dendritic cell neoplasm was usually seen in the elderly patients, mostly with the skin lesions as the first symptom. Morphological manifestations were diffuse and dense tumor cells, and single infiltration of dermis and subcutaneous tis- sues. Cells were medium size, with less cytoplasm. The nueleolus was oval or irregular, the chromatin was fine, and the nuclear fission was easy to be observed. Immunohistochemistry showed that the tumor cells positively expressed CD4, CD56, CD123; CD2, CD3, CDS, CD8, GranB, TIA-1, CD20, CD79a, PAX-5, MPO were negative. Conclusion BPDCN is a rare lymphoid hematopoietie malignancy. The clinical invasiveness is high, and it is easy to relapse, with poor prognosis, which should be differentiated with other lymphoid hematopoietic tumors.