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Title: [Clinical Features of Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm].
Author: Wu LZ, Yu Q, Jiang Y, Peng HL.
Journal: Zhongguo Shi Yan Xue Ye Xue Za Zhi; 2020 Feb; 28(1):307-313. PubMed ID: 32027294.
Abstract:
OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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. 题目: 母细胞性浆细胞样树突细胞肿瘤患者的临床特点. 目的: 分析母细胞性浆细胞样树突细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm，BPDCN)患者的临床特点、病理特征、治疗方案、治疗反应及预后，为减少临床漏诊误诊、治疗选择提供参考. 方法: 回顾性分析中南大学湘雅二医院2013年1月1日-2019年5月30日诊断治疗的9例BPDCN患者的临床、病理特征、治疗方案及疗效反应. 结果: 9例BPDCN患者最常见的受累部位是皮肤；病理特点均为真皮层受累，免疫组织化学阳性率最高的指标为CD4、CD43、LCA、CD123、VIM，均为100%；其次为CD56、 CD68、TDT、CD5、PAX-5、 CD3、CD8、CD20；9例患者MPO、EBER均为阴性。9例患者Ki-67中位数为77.5(40-90)%，这提示，CD4、CD43、LCA、CD123、VIM是诊断BPDCN敏感性最高的抗原。9例患者中2例失访，2例未接受治疗，5例接受治疗。5例中2例接受急性淋巴细胞白血病治疗方案治疗，3例接受淋巴瘤治疗方案治疗。5例患者第一周期临床症状PR 100%，其中1例患者自疾病确诊至今已6年，仍在随访. 结论: BPDCN是一种罕见且具有高度侵袭性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CD4、CD43、LCA、VIM，BPDCN接受淋系肿瘤治疗方案后有较好的应答率.

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