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Article(id=1208795422060056612, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1208795418612339683, articleNumber=null, orderNo=null, doi=10.11855/j.issn.0577-7402.2021.10.14, pmid=null, cstr=null, oa=null, hot=null, price=null, onlineType=0, articleFormat=0, articleType=null, articleTypeStr=research-article, receivedDate=1619452800000, receivedDateStr=2021-04-27, revisedDate=1626019200000, revisedDateStr=2021-07-12, acceptedDate=null, acceptedDateStr=null, onlineDate=1766128886951, onlineDateStr=2025-12-19, pubDate=1635350400000, pubDateStr=2021-10-28, doiRegisterDate=null, doiRegisterDateStr=null, onlineIssueDate=1766128886951, onlineIssueDateStr=2025-12-19, onlineJustAcceptDate=null, onlineJustAcceptDateStr=null, onlineFirstDate=null, onlineFirstDateStr=null, sourceXml=null, magXml=null, createTime=1766128886951, creator=13701087609, updateTime=1766128886951, updator=13701087609, issue=Issue{id=1208795418612339683, tenantId=1146029695717560320, journalId=1189873630562394117, year='2021', volume='46', issue='10', pageStart='955', pageEnd='1060', issueExtLink='null', onlineDate='null', pubDate='null', beforeIssueId=null, nextIssueId=null, price=null, status=1, issueComplete=1, articleOrder=1, issueType=-1, specialIssue=0, createTime=1766128886129, creator=13701087609, updateTime=1766128956061, updator=13701087609, preIssue=null, nextIssue=null, ext={EN=IssueExt(id=1208795711982924071, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1208795418612339683, language=EN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=), CN=IssueExt(id=1208795711982924072, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1208795418612339683, language=CN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=)}, issueFiles=null}, startPage=1040, endPage=1044, ext={EN=ArticleExt(id=1208795422420766780, articleId=1208795422060056612, tenantId=1146029695717560320, journalId=1189873630562394117, language=EN, title=Research progress of blastic plasmacytoid dendritic cell neoplasm, columnId=1190243275882729994, journalTitle=Medical Journal of Chinese People’s Liberation Army, columnName=Review, runingTitle=null, highlight=null, articleAbstract=

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly malignant hematological tumor derived from plasmacytoid dendritic cells (pDC) precursor cells, which has both lymphoma and leukemia characteristics, and is clinically rare. The disease easily involves the bone marrow, with low long-term survival rate and poor prognosis. Skin lesions can be seen in most BPDCN patients as the fi rst symptom, and their skin manifestations lack speci fi city, so is easy to be misdiagnosed and missed in clinic. High dose induction chemotherapy combined with hematopoietic stem cell transplantation (HSCT) are the main treatment methods for BPDCN,and new targeted therapies and immunotherapeutic drugs have also been put into clinical use. The de fi nition, epidemiology, pathogenesis,diagnosis and differential diagnosis, the latest research progress of treatment and prognosis of BPDCN have been summarized and discussed in present article, aiming to provide new ideas and references for the clinical diagnosis and treatment of BPDCN.

, correspAuthors=Jian-Zhen Shen, authorNote=null, correspAuthorsNote=
*E-mail:
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母细胞性浆细胞样树突细胞肿瘤(BPDCN)是一种来源于浆细胞样树突状细胞(pDC)前体细胞、高度恶性的血液系统肿瘤，且具有淋巴瘤和白血病的特征，临床罕见，极易累及骨髓，长期生存率低，预后差。大多数BPDCN患者以皮损为首发症状，且皮肤表现缺乏特异性，临床极易误诊、漏诊。BPDCN的治疗主要采用大剂量诱导化疗联合造血干细胞移植(HSCT)，新的靶向治疗及免疫治疗药物也已陆续投入临床使用。该文对BPDCN的定义、流行病学、致病机制、诊断和鉴别诊断、治疗最新研究进展及预后情况进行总结和论述，旨在为BPDCN的临床诊疗提供新的思路和借鉴。

, correspAuthors=沈建箴, authorNote=null, correspAuthorsNote=
沈建箴，E-mail：
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陈晓君，医学硕士，住院医师，主要从事血液肿瘤的基础与临床研究

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陈晓君，医学硕士，住院医师，主要从事血液肿瘤的基础与临床研究

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陈晓君，医学硕士，住院医师，主要从事血液肿瘤的基础与临床研究

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母细胞性浆细胞样树突细胞肿瘤的研究进展
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陈晓君 1 , 刘彦权 2 , 黄素蓉 1 , 赵立维 3 , 沈建箴 1, *
解放军医学杂志 | 综述 2021,46(10): 1040-1044
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解放军医学杂志 | 综述 2021, 46(10): 1040-1044
母细胞性浆细胞样树突细胞肿瘤的研究进展
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陈晓君1, 刘彦权2, 黄素蓉1, 赵立维3, 沈建箴1, *
作者信息
  • 1福建医科大学附属协和医院血液科淋巴瘤专科/CSCO福建抗淋巴瘤联盟，福州 350001
  • 2赣南医学院第一附属医院肿瘤科，江西赣州 341000
  • 3解放军联勤保障部队第900医院血液科，福州 350001

    • 陈晓君，医学硕士，住院医师，主要从事血液肿瘤的基础与临床研究

通讯作者:

沈建箴，E-mail：
Research progress of blastic plasmacytoid dendritic cell neoplasm
Xiao-Jun Chen1, Yan-Quan Liu2, Su-Rong Huang1, Li-Wei Zhao3, Jian-Zhen Shen1, *
Affiliations
  • 1Lymphoma Branch, Department of Hematology, Union Hospital Affiliated to Fujian Medical University/CSCO Fujian Anti Lymphoma Alliance, Fuzhou 350001, China
  • 2Department of Oncology, the First Affiliated Hospital of Gannan Medical University, Ganzhou, Jiangxi 341000, China
  • 3Department of Hematology, the 900th Hospital of the Joint Logistics Support Force of the Chinese PLA, Fuzhou 350001, China
出版时间: 2021-10-28 doi: 10.11855/j.issn.0577-7402.2021.10.14
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摘要
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母细胞性浆细胞样树突细胞肿瘤(BPDCN)是一种来源于浆细胞样树突状细胞(pDC)前体细胞、高度恶性的血液系统肿瘤，且具有淋巴瘤和白血病的特征，临床罕见，极易累及骨髓，长期生存率低，预后差。大多数BPDCN患者以皮损为首发症状，且皮肤表现缺乏特异性，临床极易误诊、漏诊。BPDCN的治疗主要采用大剂量诱导化疗联合造血干细胞移植(HSCT)，新的靶向治疗及免疫治疗药物也已陆续投入临床使用。该文对BPDCN的定义、流行病学、致病机制、诊断和鉴别诊断、治疗最新研究进展及预后情况进行总结和论述，旨在为BPDCN的临床诊疗提供新的思路和借鉴。

母细胞性浆细胞样树突细胞肿瘤  /  致病机制  /  误诊  /  治疗  /  预后

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly malignant hematological tumor derived from plasmacytoid dendritic cells (pDC) precursor cells, which has both lymphoma and leukemia characteristics, and is clinically rare. The disease easily involves the bone marrow, with low long-term survival rate and poor prognosis. Skin lesions can be seen in most BPDCN patients as the fi rst symptom, and their skin manifestations lack speci fi city, so is easy to be misdiagnosed and missed in clinic. High dose induction chemotherapy combined with hematopoietic stem cell transplantation (HSCT) are the main treatment methods for BPDCN,and new targeted therapies and immunotherapeutic drugs have also been put into clinical use. The de fi nition, epidemiology, pathogenesis,diagnosis and differential diagnosis, the latest research progress of treatment and prognosis of BPDCN have been summarized and discussed in present article, aiming to provide new ideas and references for the clinical diagnosis and treatment of BPDCN.

blastic plasmacytoid dendritic cell neoplasm  /  pathogenic mechanism  /  misdiagnose  /  treatment  /  prognosis
陈晓君, 刘彦权, 黄素蓉, 赵立维, 沈建箴. 母细胞性浆细胞样树突细胞肿瘤的研究进展. 解放军医学杂志, 2021 , 46 (10) : 1040 -1044 . DOI: 10.11855/j.issn.0577-7402.2021.10.14
Xiao-Jun Chen, Yan-Quan Liu, Su-Rong Huang, Li-Wei Zhao, Jian-Zhen Shen. Research progress of blastic plasmacytoid dendritic cell neoplasm[J]. Medical Journal of Chinese People’s Liberation Army, 2021 , 46 (10) : 1040 -1044 . DOI: 10.11855/j.issn.0577-7402.2021.10.14
正文
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母细胞性浆细胞样树突细胞肿瘤(blastic plasmacytoid dendritic cell neoplasm，BPDCN)是一种罕见的侵袭性血液系统恶性肿瘤，预后极差，好发于老年男性，中位总生存期(overall survival，OS)为1年[1-3]。BPDCN患者症状多首发于皮肤，但临床表现缺乏特异性，需与原发性皮肤科疾病、急性髓细胞白血病(acute myeloid leukemia，AML)髓外皮肤浸润、外伤引起的紫癜、髓样肉瘤和结外NK/T细胞淋巴瘤等相鉴别，其确诊通常依靠临床表现、影像学、形态学、分子生物学及免疫表型等。BPDCN的发病率低，且不易诊断，目前尚无标准的治疗方案，其治疗大致分为化学药物治疗(简称化疗)、造血干细胞移植、靶向治疗和免疫治疗。本文就BPDCN的定义、流行病学、致病机制、诊断和鉴别诊断、治疗最新研究进展及预后情况进行综述，以期为本病的临床诊疗开拓新思路。
1 概要
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BPDCN是一种罕见的高度恶性的血液系统肿瘤，来源于浆细胞样树突细胞(pDC)前体细胞，由于其在临床上极为少见，因此极易被误诊、漏诊，使患者错过最佳治疗时机。学术界在过去数十年中一直未统一该病的名称，最初被称为粒状CD4+自然杀伤(NK)细胞白血病，后被称为母细胞性NK细胞淋巴瘤及CD4+/CD56+血液皮肤肿瘤，直到2008年，世界卫生组织首次提出“母细胞性浆细胞样树突细胞肿瘤”这一名称。2008年版的《世界卫生组织造血和淋巴组织肿瘤分类》明确将BPDCN归类于AML相关的前体肿瘤，到2016年，该病被单独列为一类血液系统肿瘤[4]。BPDCN的确切发病率未知，相关研究表明其约占所有血液系统肿瘤的0.5%，发病年龄为53~70岁，男女比例为2.0~3.3:1[1-2]。值得注意的是，尽管儿童BPDCN更为罕见，但目前国外已有报道BPDCN的发病年龄呈双峰分布，其中第一高峰<20岁，第二高峰>60岁[2]。目前尚未有研究表明BPDCN存在种族易感性或地域差异性。
2 BPDCN的致病机制
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诸多研究表明，BPDCN的发病与分子生物学及遗传学改变有关，但其具体致病机制仍未明确[3-7]。从细胞遗传学及生物学角度而言，60%的BPDCN患者可出现染色体复杂核型，常见的重现型染色体异常包括5q、6q、12p、13q、15q、9号单体[4]；也有研究证实该病的发生与MYB家族基因和MYC的基因组重排有关[8]，此外相关研究表明，该病的发生涉及影响DNA甲基化或染色质重塑的表观遗传学改变[9]。最近有学者从遗传学的角度确定了髓系恶性肿瘤中常见的突变，包括RAS信号通路的激活、抑癌基因(如TP53和CDKN2A)的失活及表观遗传修饰子(如TET2)的失能[10]。此外，在BPDCN患者中可观察到t(6；8)(p21；q24)染色体易位，位于染色体6p21上的RUNX2通过调节pDC信号基因的表达而促进pDC的分化和迁移，而位于8q24上的MYC可激活现存的肿瘤细胞，因此二者共同作用促进了BPDCN细胞的增殖[10]。同样有研究发现，在缺少TET2和TP53抑癌基因的小鼠中，MYC和RUNX2的转导足以启动小鼠体内BPDCN的转化[10]。而此前也有研究报道过2例BPDCN患儿检测出t(2；8)(p12；q24)和t(X；8)(q24；q24.1)染色体易位，但成人BPDCN患者却均未检出这两种染色体易位[11]。目前，该病仍存在许多未知的致病机制，需要积累更多病例进一步研究和探索。
3 BPDCN的诊断与鉴别诊断
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3.1 诊断
BPDCN通常依靠临床表现、影像学、形态学、分子生物学及免疫表型来明确诊断。BPDCN临床表现为皮肤上的深紫色或棕色斑点、斑块，伴或不伴淋巴结大，除了侵犯骨髓和皮肤外，还可出现许多髓外病变，包括中枢神经系统、胸膜、鼻腔、睾丸、扁桃体、肺和眼睛等。其影像学表现为正电子发射计算机断层显像(PET)-CT或CT上可见淋巴结大或脏器浸润。BPDCN典型的形态学表现为中等大小的肿瘤细胞，核呈囊状圆形或不规则，通常位于周围，小核仁多见；细胞质散在弱嗜碱性颗粒，有大或小的灰色区域，形成“花岗岩”或“多云”样图案，大多数情况下没有颗粒[3]。其皮损组织病理学表现为肿瘤细胞弥漫性或致密分布于真皮及皮下组织，一般不累及表皮，肿瘤细胞灶与表皮间可见明显的无细胞浸润带(Grenz带)，无血管侵犯、坏死及炎性细胞浸润；肿瘤细胞形态单一，呈母细胞性，与原始淋巴细胞或原粒细胞相似[4,6]。其分子生物学检测结果显示可表达CD4、CD43、CD45RA和CD56，以及pDC相关抗原CD123、血树突细胞抗原2(blood dendritic cell antigen-2，BDCA-2，也称作CD303)、T细胞白血病1(T cell leukemia 1，TCL1)和溶细胞性T淋巴细胞相关抗原1(cytolytic T lymphocyte-associated antigen1，CTLA1)[5]。BPDCN的特异性免疫表型包括CD4、CD56、CD123、TCL1和CD303，通过这些特异性免疫表型基本可诊断该病。患者初次就医时应尽可能完善相关检查，包括完整的影像学资料(CT或PET-CT)以及骨髓常规、病理和流式细胞术免疫分型等[12]。通常，BPDCN的主要特征是CD123高表达、NF-κB异常活化及胆固醇代谢失调[13]。
3.2 鉴别诊断
BPDCN患者的临床症状及体征通常无特异性，或伴有皮肤受累、淋巴结大、脾大、血细胞减少和骨髓受累等，可进展为白血病[14]。该病常侵犯多个系统，大多数患者首发于皮肤，但皮肤表现缺乏特异性，易与原发性皮肤科疾病相混淆[6]。BPDCN与AML关系密切，当累及骨髓和皮肤时，可能被误诊为AML髓外皮肤浸润，不仅临床表现相似，甚至骨髓细胞形态学也难以区分；当病灶数目较少时，还可能会被误诊为外伤引起的紫癜，此时需借助骨髓流式细胞仪检测来帮助诊断及鉴别诊断。一项回顾性研究分析了90例BPDCN患者的临床表现，其中73%表现为皮肤表面有1个或几个结节，14%表现为弥散性斑块，12%表现为淤伤样斑块，但皮损严重程度与生存无关[15]。此外，BPDCN易误诊为髓样肉瘤和结外NK/T细胞淋巴瘤，详尽的免疫组织化学检查可鉴别[16]。尽管BPDCN的皮肤表现极易被误诊，但根据该病特有的形态学、分子生物学及免疫表型可进行鉴别。
4 BPDCN的治疗进展
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由于BPDCN发病率低，且不易诊断，故目前尚无标准的治疗方案，学术界将其治疗大致分为化疗、造血干细胞移植、靶向治疗和免疫治疗。
4.1 化疗
目前有研究表明，BPDCN的发病机制为表观遗传修饰基因突变和转录甲基化信号异常过表达[16]。因此，表观遗传学及基因靶向治疗很可能是一种极具潜力的治疗策略，低甲基化剂(hypomethylating agent，HMA)、阿扎胞苷和地西他滨这3种药物已引起学术界的广泛关注[7]。BPDCN最常用的治疗方案是大剂量诱导化疗。美国一项跨度13年的大型回顾性研究将化疗方案分为AML标准诱导方案、急性淋巴细胞白血病(ALL)标准诱导方案、CHOP样方案、大剂量甲氨蝶呤联合天冬酰胺酶(Aspa-MTX)化疗方案及其他治疗方案，结果显示，接受AML及ALL标准诱导方案或Aspa-MTX化疗方案的患者，其生存期长于接受CHOP样方案或其他治疗方案的患者[3]。值得注意的是，BPDCN易侵犯中枢神经系统。有研究表明，BPDCN患者若出现中枢浸润，则复发频率增加，如果化疗方案未覆盖中枢神经系统，则需加强中枢浸润预防[17]，推荐预防性鞘内注射化疗药，或使用其他可透过血脑屏障的药物如塞替派等。目前国际上推荐的低强度化疗药有苯达莫司汀、地西他滨、吉西他滨+多西他赛等。其中，苯达莫司汀具有烷基化和抗代谢物结构的功能及特性，可抑制有丝分裂突变且与其他烷基化剂无交叉抵抗性。一项研究报道了5例复发难治的BPDCN患者使用苯达莫司汀单药治疗的效果，结果显示，其中1例获得了持续7个月的完全缓解(complete remission，CR)[18]，尽管并未达到令人满意的程度，但可进一步研究苯达莫司汀用于复发难治BPDCN患者的疗效。吉西他滨在多种复发难治性淋巴瘤中均表现出一定疗效，且不良反应小，而多西他赛对肿瘤细胞具有细胞毒性，因此，吉西他滨+多西他赛也被用于复发难治BPDCN患者的治疗。Ulrickson等[19]采用吉西他滨+多西他赛治疗3例复发难治BPDCN患者，结果显示，该方案的总体耐受性良好，2例达到了CR，且中位OS为13.3个月。由此可见，吉西他滨+多西他赛治疗BPDCN也是一种可行的化疗方案。目前，针对BPDCN患儿的治疗经验非常罕见且缺乏前瞻性临床试验验证，现有的结果表明，BPDCN患儿使用ALL标准诱导方案并辅以中枢预防的疗效较好[20]。通常复发或难治性BPDCN的预后极差，可选择的治疗方案更是有限，对于诱导化疗后复发的BPDCN患者，建议基于其身体状况更换另一种方案[21]。单纯诱导化疗CR后疗效不持久，复发时间短。此外，首次缓解时进行造血干细胞移植(HSCT)可提高总体生存率[3]。
4.2 HSCT
除小儿BPDCN病例外，单靠化疗获得缓解的可能性较低，因此HSCT对首次获得CR的患者维持长期生存显得尤为重要。
异基因造血干细胞移植(Allo-HSCT)是治愈多种血液系统肿瘤的有效手段，且已成为达到CR的BPDCN患者的标准治疗方法，但目前仍无将Allo-HSCT与常规化疗或新疗法进行比较的随机对照试验。一项北美的多中心观察性研究评估了Allo-HSCT对37例BPDCN患者的疗效，结果显示，3年总体生存率和无进展生存率分别为58%和55%，首次缓解后进行移植的患者，其3年总体生存率和无进展生存率更高，分别为74%和69%[22]。2013年欧洲血液和骨髓移植组(European Blood and Bone Marrow Transplantation Group，EBMT)的数据显示，接受Allo-HSCT的34例BPDCN患者中有16例存活，中位生存时间为28(4~77)个月，3年非复发死亡发生率、无病生存率和总体生存率分别为30%、33%和41%；有11例(32%)复发，中位复发时间为8(2~27)个月，未发现晚期复发(>27个月)的患者，表明Allo-HSCT治疗BPDCN可能已达到持久缓解甚至根治[23]。Cernan等[24]对捷克共和国2000—2017年诊断为BPDCN的14例患者进行了回顾性分析，其中男10例，女4例，中位年龄39(5~68)岁，9例接受了Allo-HSCT，其中2例在异基因移植后即获得首次完全缓解，但接受Allo-HSCT的患者总生存时间长于未接受移植的患者(中位生存时间分别为16.4个月、8.1个月)。一项纳入接受Allo-HSCT(n=37)或自体造血干细胞移植(Auto-HSCT，n=8)治疗的45例患者的研究发现，无论年龄、移植前治疗或移植缓解状态如何，Allo-HSCT的疗效均优于Auto-HSCT，尤其对于首次完全缓解(CR1)的患者[25]。
Auto-HSCT在BPDCN中的作用评估数据目前仅限于单个病例报告或小型研究，如Kharfan-Dabaja等[22]收集了2003—2013年接受Auto-HSCT治疗的11例BPDCN患者的相关数据，中位年龄为57岁，结果显示4年总体生存率为82%；其中4例在首次CR或部分缓解(partial remission，PR)时接受了Auto-HSCT治疗，最终3例死亡或复发。由于接受Auto-HSCT治疗的BPDCN病例数少且疗效无法评估，因此未将Auto-HSCT推荐为BPDCN的标准治疗方法[22]。鉴于目前研究认为Allo-HSCT比Auto-HSCT治疗更有效，因此笔者建议接受其他方案治疗的BPDCN患者，首次获得CR后应及早进行Allo-HSCT。
4.3 靶向治疗
对于不适于用高强度诱导化疗及HSCT方案的老年患者，除采用低强度化疗之外，靶向治疗也是一种可供选择的治疗方案。CD123是Ⅰ型跨膜糖蛋白，与CD131共同形成二聚体，参与IL-3信号转导，由于其在AML和几乎所有BPDCN患者体内高表达，因此成为了BPDCN的一个潜在治疗靶点。Tagraxofusp是近几年才开始用于治疗BPDCN的一种CD123靶向药，美国一项多队列研究对47例未经治疗或复发的患者静脉注射7或12 μg/kg的Tagraxofusp(连续给药5 d，每3周为1个疗程)，持续治疗直至疾病进展或产生不可接受的不良反应，其中29例未经治疗的患者静脉注射12 μg/kg Tagraxofusp后CR率达72%，研究期间出现的严重不良反应包括毛细血管渗漏综合征、肝功能障碍和血小板减少症[1]。Tagraxofusp安全性尚可，且其不良反应总体上可接受，已被美国食品药品监督管理局(FDA)批准上市[1]。
目前，去甲基化药物阿扎胞苷已被批准用于中高危骨髓增生异常综合征(myelodysplastic syndromes，MDS)和AML的治疗，而BPDCN也是一种髓样恶性肿瘤，部分可转化为AML或合并MDS[6,26]，且在BPDCN患者中也检测到DNA甲基化基因TET2的突变，因此阿扎胞苷也被用于该病的治疗。有研究评估了3例BPDCN患者使用阿扎胞苷化疗的预后情况，结果显示患者的皮肤病变均得到改善，其中1例达到持久缓解，所有患者耐受性均较好，但中位生存期仅17个月[26]。
Bcl-2抑制剂是近年才开始应用于临床的一种针对B细胞淋巴瘤的靶向治疗药，同样可用于BPDCN患者的治疗。Bcl-2基因可编码抑制细胞凋亡的Bcl-2蛋白，其在BPDCN中呈过表达。有研究通过直接细胞毒性评估、细胞凋亡测定和动态Bcl-2同源结构域3(Bcl-2 homology domains 3，BH3)分析等发现，BPDCN肿瘤细胞对Bcl-2抑制剂Venetoclax敏感。国外一项研究报道了1例复发/难治性BPDCN患者接受Venetoclax单药治疗6周后，皮损大面积消退，PET-CT示多发肿大淋巴结较前显著缩小，骨髓中BPDCN肿瘤细胞从治疗前的85%减少到44%，因此推荐Venetoclax或其他Bcl-2抑制剂单独或联合其他疗法用于BPDCN患者的治疗[27]。
此外，也有研究表明，BET(bromodomain and extra-termina)1抑制剂对BPDCN肿瘤细胞具有高度毒性，T细胞因子4(T cell factor，TCF4)作为BPDCN中的关键癌基因，被当作BET抑制剂靶向治疗的一个靶点[28]。目前，临床上正在开发BET抑制剂单药或联合药物，但尚未明确BPDCN肿瘤细胞上是否存在对BET抑制剂高度敏感的特异性靶点[29]。
4.4 免疫治疗
嵌合抗原受体T细胞(CAR-T)疗法是近年来新兴的免疫疗法，在多种肿瘤的治疗中效果显著。CD123在BPDCN中高表达，逆转录病毒或具有第三代CAR的慢病毒可识别CD123，而CD123 CAR特定的单链抗体(scFV)具有较高的亲和力，可与BPDCN患者的肿瘤细胞相结合，而不与血液或骨髓中的正常细胞结合，从而发挥精准杀灭BPDCN肿瘤细胞的作用。CAR-T治疗的常见不良反应是细胞因子释放综合征(CRS)。有研究发现，单核细胞是IL-1和IL-6的主要来源，而单核细胞表达低水平的CD123，当CD123 CAR-T细胞在体内攻击单核细胞时可能会抑制CRS的发生[30]，因此，CD123 CAR-T疗法的CRS发生率较其他CAR-T疗法低，在BPDCN的治疗中具备一定的可行性和安全性。
程序性死亡配体-1(programmed cell death ligand-1，PD-L1)表达于肿瘤细胞和抗原呈递细胞，通过与T细胞上的程序性死亡受体-1(programmed cell death protein-1，PD-1)相互作用而抑制T细胞的活化。Nivolumab是一种抗PD-1单克隆抗体，可阻止T细胞上的PD-1与肿瘤细胞上的PD-L1结合，从而使人的免疫系统释放相关物质以破坏肿瘤细胞。最近，Nivolumab已被FDA批准用于转移性黑色素瘤、肾细胞癌、小细胞肺癌和经典霍奇金淋巴瘤的临床试验，目前的Ⅱ期临床试验正在观察外周T细胞淋巴瘤和BPDCN患者对该药的反应[31]。
5 BPDCN的预后情况
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BPDCN是一种罕见的侵袭性造血系统肿瘤，中位生存期<2年(17~34个月)[32]。Kim等[33]的一项回顾性研究比较了BPDCN儿童患者与成人患者的预后，结果证实年龄是其独立预后因素，儿童BPDCN在临床上的恶性程度较成人低，通常预后尚可，而成人患者则预后极差。尽管大多数患者对各种化疗方案均有反应，但极易复发，且复发后治疗效果较差，其OS仅为1年[3]。年龄>60岁、染色体核型异常和BPDCN细胞的末端脱氧核苷酸转移酶(TdT)阴性均预示患者预后较差[34]。
6 总结与展望
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BPDCN是一种罕见的来源于pDC前体细胞的血液系统恶性肿瘤，大多数患者首发症状表现为皮损，误诊率高，确诊后应尽快治疗以改善预后。治疗上需根据患者年龄、发病部位、病理类型、累及范围、分期、免疫表型和分子生物学特性等制定个体化的治疗方案，以延长患者的生存时间。BPDCN的治疗大致分为化疗、HSCT、靶向治疗和免疫治疗，虽然目前尚缺乏最佳治疗方案与共识，但经其他方案治疗后首次获得CR时行Allo-HSCT可能是未来初诊BPDCN患者的主要选择。考虑到BPDCN的发病率相对较低，目前仍需积累更多病例，开展更为深入的研究，未来应重点探索其分子生物学特点与临床特征的关系，以期达到更精准的治疗，从而更有效地提高BPDCN患者的长期生存率。
基金
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  • 国家自然科学基金(81300428)
文献
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参考文献 引证文献
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2021年第46卷第10期
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doi: 10.11855/j.issn.0577-7402.2021.10.14
  • 接收时间：2021-04-27
  • 首发时间：2025-12-19
  • 出版时间：2021-10-28
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  • 收稿日期：2021-04-27
  • 修回日期：2021-07-12
基金
National Natural Science Foundation of China(81300428)
国家自然科学基金(81300428)
作者信息
    1福建医科大学附属协和医院血液科淋巴瘤专科/CSCO福建抗淋巴瘤联盟，福州 350001
    2赣南医学院第一附属医院肿瘤科，江西赣州 341000
    3解放军联勤保障部队第900医院血液科，福州 350001

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2种不同金属材料的力学参数
科
Family		 属数
Number of
genus		 种数
Number of
species		 占总种数比例
Percentage of
total species (%)		 属
Genus		 种数
Number of
species		 占总种数比例
Percentage of total
species (%)
鹅膏菌科Amanitaceae 2 11 5.26 鹅膏菌属 Amanita 10 4.78
小菇科 Mycenaceae 2 12 5.74 丝盖伞属 Inocybe 5 2.39
多孔菌科 Polyporaceae 8 14 6.70 蜡蘑属 Laccaria 5 2.39
红菇科 Russulaceae 3 23 11.00 小皮伞属 Marasmius 6 2.87
小菇属 Mycena 11 5.26
光柄菇属 Pluteus 5 2.39
红菇属 Russula 17 8.13
栓菌属 Trametes 5 2.39
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