母细胞性浆细胞样树突细胞肿瘤1例报告并文献复习

BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASM:A CASE REPORT AND REVIEW OF LITERATURE

目的提高对母细胞性浆细胞样树突细胞肿瘤(BPDCN)的认识。方法报告1例BPDCN病儿临床资料并进行文献复习。结果该病儿以皮肤起病,累及骨髓及淋巴结,肿瘤细胞表达CD56、CD123、BDCA-4等特异性标记,VDLD方案可诱导缓解,异基因造血干细胞移植治疗该病有效。结论 BPDCN非常罕见,呈高度侵袭性,预后差,发病机制尚不明确,诊断依赖于临床资料、组织病理学和免疫表型标记,目前尚无标准治疗方案。

Objective To enhance the understanding of blastic plasmacytoid dendritic cell neoplasm（BPDCN）. Methods Clinical information of one child with BPDCN was reported and literature concerned reviewed. Results The lesion in the child started from skin,and bone marrow and lymph nodes were involved.The tumor cells expressed specific markers as CD56,CD123and BDCA-4.VDLD chemotherapy could quickly induce remission,and allogeneic hematopoietic stem cell transplantation treatment proved effective. Conclusion Blastic plasmacytoid dendritic cell neoplasm is an extremely rare and highly aggressive hematological malignancy with poor prognosis,and its pathogenesis is still unclear.The diagnosis of this disease is mainly based on clinical information,histopathologic and immunophenotype markers.A standard treatment protocol for this malignancy has not yet been established.