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

摘要

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.