| 曹春育,陈丽华,戴桂红.母细胞性浆细胞样树突细胞肿瘤 2例临床病理研究[J].安徽医药,2023,27(9):1861-1864. |
| 母细胞性浆细胞样树突细胞肿瘤 2例临床病理研究 |
| Two cases of maternal plasmacytoid dendritic cell tumors: a clinicopathological study |
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| DOI：10.3969/j.issn.1009-6469.2023.09.037 |
| 中文关键词: 母细胞性浆细胞样树突细胞肿瘤 抗原， CD 免疫表型 临床病理特征 |
| 英文关键词: Blastic plasmacytoid dendritic cell neoplasm Antigens, CD Immunophenotype Clinicopathologic features |
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| 中文摘要: |
| 目的探讨母细胞性浆细胞样树突细胞肿瘤( BPDCN)的临床病理形态特征及诊断与鉴别诊断。方法对泰州市人民医院 2020年住院及 2019年会诊的 2例 BPDCN的临床表现、组织病理学形态及免疫表型进行观察和分析，并复习相关文献。结果 2例病人均为男性，年龄 76岁和 42岁，前者表现为面部单发皮肤结节，后者表现为左颈部淋巴结肿大。镜下，淋巴结内或皮肤真皮及皮下组织见弥漫一致肿瘤细胞浸润，肿瘤细胞中等大小，细胞核卵圆形或不规则，染色质细腻，可见小核仁，胞质稀少，呈母细胞样。 2例病人的肿瘤细胞均表达白细胞分化抗原 4(CD4)、白细胞分化抗原 56(CD56)和白细胞分化抗原 123(CD123)，均不表达淋系、髓系及自然杀伤细胞特异性标记，原位杂交检测 EB病毒编码的小 RNA(EBER)阴性。结论 BPDCN是一种少见的高度侵袭性的淋巴造血系统恶性肿瘤，预后差，易累犯皮肤，其诊断需结合临床表现、组织学形态及免疫表型综合分析。 |
| 英文摘要: |
| Objective To discuss the clinicopathologic-morphologic features and diagnosis and differential diagnosis of blastic plas- macytoid dendritic cell neoplasm (BPDCN).Methods The clinical manifestations, histopathologic morphology, and immunophenotypewere observed and analyzed in two cases of BPDCN hospitalized in Taizhou People's Hospital in 2020 and consulted in 2019, and therelated literature was reviewed.Results Both patients were male, aged 76 and 42 years old. The former presented with a single facialskin nodule, while the latter showed an enlarged lymph node in the left neck. Microscopically, the lymph nodes or the dermal and sub-cutaneous tissues of the skin were infiltrated by diffuse and uniform tumor cells, which were medium-sized, with ovoid or irregular nu-clei, fine chromatin, small nucleoli, sparse cytoplasm, and a matricellular appearance. Tumor cells from both patients expressed leuko-cyte differentiation antigen 4 (CD4), leukocyte differentiation antigen 56 (CD56), and leukocyte differentiation antigen 123 (CD123),and none of them expressed gonadal, myeloid, or natural killer cell-specific markers. In situ hybridization was negative for small RNA encoded by EBV (EBER).Conclusion BPDCN is a rare and highly invasive lymphohaematopoietic malignant tumor with poor progno-sis and easy involvement of the skin, and its diagnosis depends upon a comprehensive analysis of clinical manifestations, histologicalmorphology, and immunophenotype. |
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