母细胞性浆细胞样树突细胞肿瘤41例临床分析  

Clinical Analysis on 41 Cases of BPDCN

作　　者：饶进[1] 殷莉[1] 林志美[1] 李世云[1] 熊萍[1] 

机构地区：[1]成都大学附属医院血液内科,成都610081

出　　处：《临床误诊误治》2014年第12期25-28,共4页Clinical Misdiagnosis & Mistherapy

摘　　要：目的总结母细胞性浆细胞样树突细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm,BPDCN)的诊治要点及预后,为临床进一步认识和诊治该病提供依据。方法以"母细胞性浆细胞样树突细胞肿瘤"、"母细胞性NK细胞淋巴瘤/白血病"或"无颗粒型CD4+CD56+血液皮肤肿瘤"及"BPDCN"为检索词,检索2009年1月—2013年12月万方数据库、中国知网(CNKI)和Pub Med发表的相关病例报道并对其资料进行回顾性分析。结果经检索共筛选出26篇文献BPDCN 41例,男29例,女12例;平均63.7岁。临床主要表现为皮肤受损伴淋巴结和骨髓受累,肿瘤细胞表达CD4、CD56、CD123,不表达髓系、T细胞及B细胞特异性标志。本组41例采用淋巴瘤、急性髓系白血病、急性淋巴细胞白血病方案治疗或单药化疗,其中33例达血液学缓解,有20例完全缓解;15例在化疗后予异体移植,其中14例达血液学缓解。41例平均生存期为12.3个月。结论 BPDCN以老年人好发,诊断依赖于临床资料、组织病理学和免疫组织化学标记,病程呈侵袭性,预后多较差。Objective To investigate the clinical diagnosis, treatment and prognosis of BPDCN, and to improve the understanding and treatment of BPDCN. Methods Literatures on BPDCN, blastic natural-killer-cell lymphoma or CD4 ＋CD56＋ haematodermic neoplasm published in journals between January 2009 and December 2013 were identified by searching Wanfang, PubMed and CNKI. A retrospective review was carried out. Results A total of 41 BPDCN patients were found from the 28 literatures, including 29 males, 12 females;the median age was 63. 7 years. Major clinical manifestations were skin lesions and often involving lymph nodes and bone marrow. The tumors were CD4 ＋, CD56 ＋ and CD123 ＋. Lineage specific markers for B-and T-cell were negative and the tumors did not express myelopemxidase. 33 of the 41 patients accepted lymphoma, acute myeloid leukemia （ AML） , acute Lymphocytic leukemia-like chemothpy and single agent chemotherapy at-tained hematological remission, and 20 patients obtained complete remission. 15 patients were treated with allogeneic SCT, and 14 of the 15 patients obtained hematological remission. The median overall survival （ MS） time of 41cases was 12. 3 months. Conclusion BPDC hematodermic neoplasm is a rare hematopoietic malignancy, which primarily affects elderly pa-tients. The diagnosis of this disease is mainly based on the clinical presentations, pathologic and immunohistochemical fea-tures. Clinical course is rapidly and fatally aggressive. Patients with BPDCN usually have a poor prognosis.

关 键 词：母细胞性浆细胞样树突细胞肿瘤 皮肤表现 免疫表型分型 治疗结果 预后 

分 类 号：R733[医药卫生—肿瘤]