母细胞性浆细胞样树突细胞肿瘤研究进展

The Research Progress of Blastic Plasmacytoid Dendritic Cell Neoplasm

2008年WHO在造血和淋巴系统肿瘤分类中将发生于皮肤,CD56和CD4阳性,并表达CD123,血树突细胞抗原2和4等浆细胞样树突细胞标记的所谓"母细胞性NK细胞淋巴瘤"命名为母细胞性浆细胞样树突细胞肿瘤,认为其起源于浆细胞样树突状细胞,是一种罕见的临床侵袭性淋巴瘤,可发生于各年龄组,多数为中老年男性。临床多有皮肤损害,表现为孤立性、多发性肿块、结节、斑块、红斑,可迅速累及淋巴结、软组织和中枢神经系统,临床过程凶险。组织学有一定特点,确诊依赖对肿瘤细胞的免疫标记。患者最初对化疗可有较好反应,但复发快,复发率高,中位生存时间仅12～14个月。

It was named as the blastic plasmamacytoid dendritic cell neoplasm in the 2008 by WHO in classification of tumors of hematopoietic and lymphatic systems,that occured in the skin, CD56 and CD4 positive,and ex- pressed CD123,blood dendritic cell antigen 2 and 4,plasma cells and other dendritic cell markers positive, and called blastic NK cell lymphoma. Blastic plasmamacytoid dendritic cell neoplasm originates in the plas- mamacytoid dendritic cells,is a rare and clinically aggressive lymphoma. The disease can occur in all age groups, but mostly in middle-aged men. Clinically, most patients have skin lesion, which is solitary or multi- ple lumps,nodules,plaques,erythema,and rapidly involving the lymph nodes,soft tissue and central nervous system.The disease develops quickly and the prognosis is poor. Although histology is characteristic,but di- agnoses of blastic plasmamacytoid dendritic cell neoplasm must depend upon the immunohistochemistry. Patients may have a better response to chemotherapy at first, but the disease relapses quickly and has a high recurrence rate. The median survival time of patients is only 12 to 14 months.