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目的探讨母细胞性浆细胞样树状突细胞肿瘤(BPDCN)的组织病理学特点、诊断与鉴别诊断。方法对1例病理会诊BPDCN患者的组织标本进行HE染色、常规Eli Vision免疫组化染色及原位杂交检测,并结合文献复习分析其临床病理特点。结果患儿男性,10岁。以头颈部包块就诊。镜下瘤细胞形态类似于淋巴母细胞或原始单核细胞。免疫组化示瘤细胞CD4、CD56、CD43和CD123(+),CD34、CD117、MPO、CD68、CD20和CD3(-)。结论 BPDCN是一种罕见类型高度侵袭性的血液肿瘤,进展快,病死率高,易误诊。诊断依据临床和组织学特点,结合免疫组化标记,应注意与急性白血病和其他类型淋巴瘤区别。
Objective To investigate the histopathological features, diagnosis and differential diagnosis of the somatic cell-like plasmacytoid dendritic cell tumor (BPDCN). Methods Tissue samples from 1 patient with BPDCN diagnosed by pathology were examined by HE staining, routine Eli Vision immunohistochemical staining and in situ hybridization. The clinical and pathological features were reviewed according to the literature review. Results children male, 10 years old. Head and neck mass treatment. Microscopic tumor cells resemble lymphoblast cells or primitive mononuclear cells. Immunohistochemistry showed tumor cells CD4, CD56, CD43 and CD123 (+), CD34, CD117, MPO, CD68, CD20 and CD3 (-). Conclusion BPDCN is a rare type of highly aggressive hematologic tumor with rapid progression and high mortality. Diagnosis based on clinical and histological features, combined with immunohistochemical markers, should pay attention to acute leukemia and other types of lymphoma difference.