母细胞性浆细胞样树突细胞肿瘤

Blastic plasmacytoid dendritic cell neoplasm

母细胞性浆细胞样树突细胞肿瘤是一种少见的侵袭性淋巴瘤,2008年正式命名.临床上以皮肤受累为首发症状,表现为挫伤样结节或斑块.该肿瘤易侵犯骨髓及淋巴结,病程凶险,预后差.其肿瘤细胞来源于浆细胞样树突细胞前体细胞,具有独特的免疫表型：CD4、CD56阳性,TdT和CD68部分阳性,还表达浆细胞样树突细胞相关表面标志CD123、TCL1和BDCA-2.但目前其发病机制仍未明确,且无标准治疗方案.

Blastic plasmacytoid dendritic cell neoplasm （BPDCN） is a rare invasive lymphoma, which was recognized as an independent entity in the 2008 World Health Organization （WHO） classification of cutaneous lymphomas. Cutaneous involvement often represents the first manifestation of this disease, which may appear as bruise-like nodules or plaques. It has a predilection for the invasion of bone marrow and lymph nodes with an aggressive course and poor prognosis. The tumor cells , which have been considered to derive from the precursors of plasmacytoid dendritic cells with special immunophenotype, are positive for CD4, CD56,plasmacytoid dendritic cell-associated antigens （CD123, TCL1 and BDCA-2）, and partly positive to TdT and CD68. Currently, the aetiology of BPDCN remains unknown and there are no standard therapeutic schemes.