母细胞性浆细胞样树突状细胞瘤

Blastic plasmacytoid dendritic cell neoplasm

本文报告母细胞性浆细胞样树突状细胞瘤(BPDCN)1例。患者男,70岁。反复皮肤瘙痒伴红斑结节1年。皮损组织病理:瘤细胞在真皮内密集,呈结节、团块状浸润,核深染,形态不规则,可见病理性核分裂象。免疫组化:CD4、CD56、CD123、CD43、CD45RA均阳性。骨髓涂片:骨髓增生明显活跃,异常细胞占72.8%,骨髓流式免疫分型:人白细胞DR抗原(HLA-DR):98.6%,CD56:82.3%,CD38:83%,CD4:92%和CD123:94.6%。该文对BPDCN的诊断、鉴别诊断和治疗进行讨论,揭示该病呈高度侵袭性,预后差,生存期短。

Blastic plasmacytoid dendritic cell neoplasm （BPDCN） is a rare clinically aggressive tumor derived from the precursors of plasmacytoid dendritic cells, with high frequency of cutaneous involvement as the initial manifestation. A case of BPDCN is reported. A 70-year-old man presented with repeated pruritic erythema and nodules for 1 year. Histopathological examination indicated that the tumor cells infiltrated in the dermis in the form of nodule or mass. The nuclei of the tumor cells were darkly stained with irregular shape, and atypical karyokinesis was observed. Immunohistoehemisty revealed that CD4,CD56,CD123,CD43 and CD45RA were positive. Bone marrow smear showed active proliferation and abnormal blastic cells accounted for 72.8%. It was confirmed by flow cytometry that the blastic cells in the the bone marrow expressed HLA- DR（98.6%）, CD56（82.3%）, CD38（83%）, CD4（92%） and CD123 （94.6%）. The diagnosis, differential diagnosis and therapy of BPDCN were discussed, indicating BPDCN is an aggressive disease with poor prognosis and short survival time.