Wu Ling-Zhen, Yu Qian, Jiang Yi, Peng Hong-Ling
Department of Hematology, The Second Xiangya Hospital, Central South University, Changsha 410011, Hunan Province, China.
Department of Hematology, The Second Xiangya Hospital, Central South University, Changsha 410011, Hunan Province, China,E-mail:
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2020 Feb;28(1):307-313. doi: 10.19746/j.cnki.issn.1009-2137.2020.01.051.
To analyze the clinical characteristics, pathological features, treatment response and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN), so as to provide reference for reducing clinical misdiagnosis and treatment regimens.
The clinical, pathological features, treatment regimens and treatment response of 9 patients with BPDCN diagnosed and treated from January 1, 2013 to June 30,2019 in Xiangya Second Hospital of Central South University were analyzed respectively.
The most common site of involvement in 9 patients with BPDCN was skin; the pathological features were involvement of dermis, and the highest positive immunohistochemical indexes rate were CD4, CD43, LCA, CD123, and VIM (all 100%), followed by CD56 and CD68, TDT, CD5, PAX-5, CD3, CD8, CD20; MPO and EBER of 9 patients were negative. However, Ki-67 of the 9 patients showed a medican of 77. 5% (40%-90%), which suggests that CD4, CD43, LCA, CD123, and VIM were the most sensitive antigens for diagnosing BPDCN. Of the 9 patients, 2 cases were lost to follow-up, 2 cases were untreated, and 5 cases were treated, in which 2 cases received treatment of regiment for acute lymphoblastic leukemia and 3 cases received treatment of regimen for lymphoma. The PR of clinical symptoms in the first cycle in 5 patients were 100%, and 1 patient has survived for 6 years since the disease was diagnosed.
BPDCN is a rare and highly invasive lymphoid hematopoietic malignancy. The most common initial symptom of the patients is skin lesions. The most sensitive pathological indicators are CD4, CD43, LCA, VIM, and the BPDCN patients received treatment of regimens for lymphoid tumor show a better response rate.
分析浆细胞样树突状细胞肿瘤（BPDCN）患者的临床特征、病理特点、治疗反应及预后，为减少临床误诊及制定治疗方案提供参考。
对2013年1月1日至2019年6月30日在中南大学湘雅二医院确诊并治疗的9例BPDCN患者的临床、病理特征、治疗方案及治疗反应进行分析。
9例BPDCN患者最常见的受累部位为皮肤；病理特征为真皮受累，免疫组化指标阳性率最高的是CD4、CD43、LCA、CD123和VIM（均为100%），其次是CD56和CD68、TDT、CD5、PAX - 5、CD3、CD8、CD20；9例患者的MPO和EBER均为阴性。然而，9例患者的Ki - 67中位数为77.5%（40% - 90%），提示CD4、CD43、LCA、CD123和VIM是诊断BPDCN最敏感的抗原。9例患者中，2例失访，2例未治疗，5例接受治疗，其中2例接受急性淋巴细胞白血病方案治疗，3例接受淋巴瘤方案治疗。5例患者第一周期临床症状的PR为100%，1例患者自确诊疾病后已存活6年。
BPDCN是一种罕见且侵袭性高的淋巴造血恶性肿瘤。患者最常见的首发症状是皮肤病变。最敏感的病理指标是CD4、CD43、LCA、VIM，接受淋巴瘤方案治疗的BPDCN患者显示出较好的反应率。
