母细胞性浆细胞样树突状细胞肿瘤一例并文献复习

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1、万方数据·290·自堕疸：鲞旦疸!Q!!生!旦箜丝鲞箜!塑』业里堂!!堡!!!竺堕垦!Y里P!!里!!丛型!Q!!!!!!：丝：№：!母细胞性浆细胞样树突状细胞肿瘤一例并文献复习周继豪孙璇林海清孙雄飞姜容张新友【摘要】目的提高对母细胞性浆细胞样树突状细胞肿瘤(BPDCN)的认识。方法通过1例病例报告和文献复习，分析BPDCN患者的临床特点、治疗选择及预后。结果BPDCN患者以中老年男性居多，绝大多数发病时表现为皮疹和骨髓浸润，特征性免疫表型为CD4+,CD56+,CDl23+。在诱导治疗方面，淋系方案比髓系方案治疗的总反

2、应率高，复发率较低，总生存较长。异基因造血干细胞移植可能带来长期生存。患者发病时白细胞和血小板总数可能是影响BPDCN预后的危险因素。结论BPDCN在临床表现和免疫表型上都具有显著特点，治疗方案建议以淋系方案为主，在首次完全缓解期行异基因造血干细胞移植是改善预后、获得远期生存的重要手段。【关键词】树突状细胞肿瘤；母细胞；浆细胞样树突状细胞Blasticplasmacytoiddendriticcellneoplasm：acasereportandliteraturerenewZhou正^∞，SunXuan,LinH叻in

3、g,SunXiongfei，JiaugRong,ZhangXinyou．DepartmentofHematology,theSecondClinicalMedicalCollegeofJinanUniversity,ShenzhenPeople’sHospital，Shenzhen518000,ChinaCorrespondingauthor：ZhangXinyou，Email：zxy0518@live．corn【Abstract】ObjectiveToprovidenewinformationfortreatmenta

4、ndprognosisofblasticplasmacytoiddendriticcellneoplasm(BPDCN)．MethodsThroughonecasereportandliteraturereviewof48BPDCNcaseswerereviewedretrospectively．Theclinicalcharacteristics，treatmentchoicesandprognosiswereanalyzed．ResultsBPDCNpatientsweremainlyelderlymales，mos

5、tlypresentedasskinrashandbonemarrowinfiltration．ImmunophenotypewascharacteristicallyexpressedasCD4，CD56andCD123．Lymphoid-likeregimenscouldinducehigherresponserate，lowerrelapserateandlongeroverallsurvivalcomparedwithmyeloid—likeregimens．Allogeneichematopoieticstem

6、celltransplantationmayprovidelong—termsurvival．Attheonsetofthedisease，Thecountsofwhitebloodcells(WBC)andbloodplatelet(Pit)maybecorrelatedwithinferioroverallsurvival．ConclusionsBPDCNiSadiseasewithdistinctclinicalcharacteristicsandimmunophenotype．Lymphoid—likeregim

7、enmaybethebettertreatmentofchoice，whileallogeneichematopoieticstemcelltransplantationshouldbetakenintoaccountinthefirstcompleteremissionforlong—termsurvival．【Keywords】Dendriticcellneoplasm；Blastic；Plasmacytoiddendriticcells母细胞性浆细胞样树突状细胞肿瘤(blasticplasmacytoiddendr

8、iticcellneoplasm，BPDCN)是一种罕见的血液系统恶性肿瘤，临床进程高度侵袭，易侵犯皮肤和骨髓，免疫表型表现为特异性的CD4+、CD56+,CDl23+。2008年WHO淋巴和血液系统疾病分类中首次将BPDCN列为独立的疾病实体[1]。由于本病发病率低，预后差，多为散在病例报道，缺少大规模的临床研究资