母细胞性浆细胞样树突细胞肿瘤41例临床分析

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。

Clinical Analysis on 41 Cases of BPDCN

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.