33例中国地区母细胞性浆细胞样树突细胞肿瘤患者临床资料分析

Clinical characteristics of 33 cases of blastic plasmacytoid dendritic cell neoplasm in China

目的 分析母细胞性浆细胞样树突细胞肿瘤（BPDCN）的临床特点及治疗效果.方法 回顾性分析33例中国地区确诊BPDCN的临床病例资料.结果 共收集23篇文献,包括患者33例,男性23例,女性10例,中位年龄50岁,主要临床表现为皮肤病变,可合并骨髓、外周血或淋巴结受累.免疫表型提示BPDCN细胞多表达CD4为90.0%（27/30）,CD56为100%（33/33）,CD123为100%（28/28）,TCL-1为87.5%（7/8）,CD43为94.4%（17/18）;而CD3,CD20,CD79a,MPO阳性表达率较低.应用急性白血病或淋巴瘤样治疗方案,疗效不佳,患者生存期短,中位生存仅8个月,95%置信区间为3.508-12.492.结论 BPDCN为血液系统少见的恶性肿瘤,有特征性的免疫病理学标记,预后差,需要更多样本的资料来加强对疾病认识进而提高疗效.

Objective To analyze the clinical characteristics and treatment of 33 Chinese patients with blastic plasma-cytoid dendritic cell neoplasm （BPDCN）. Method A retrospective study was conducted focusing on the clinical data of 33 Chinese patients with BPDCN. Result A total of 23 literatures were selected, including 33 patients with BPDCN, in which there were 23 male and 10 female cases;the median age was 50 years. The major clinical manifestation was skin le-sions and usually involving bone marrow, peripheral blood and lymph nodes. The results of immuophenotyping showed a high expression rate of CD4 （90.0%, 27/30）, CD56 （100%, 33/33）, CD123 （100%, 28/28）, TCL-1 （87.5%, 7/8） and CD43 （94.4%, 17/18）. However, some specific B-, T-cell or myeloid markers hardly expressed, such as CD3, CD20, CD79a and MPO. Patients in the collected case reports accepted acute myeloid leukemia （AML）, acute lymphocytic leukemia （ALL） and lymphoma-like chemotherapy showing poor treatment effects. The total survival period is very short, with a median overall survival time being only 8 months （95%CI, 3.508-12.492） in this study. Conclusion BPDCN is a rare and ag-gressive hematopoietic malignancy with a poor prognosis. Patients with BPDCN usually have specific clinical presenta-tions and immunohistochemical features. Large sample researches are needed to improve the understanding and treatment of BPDCN.