慢性移植物抗宿主病.ppt

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1、ChronicGVHD:PathophysiologyandNovelTherapeuticStrategiesTingLiuDepartmentofHematologyWestChinaHospitalSichuanUniversity2014.4.Xiamen内容UpdateofknowledgesincGVHDProgressinpathophysiologyofcGVHDTreatmentforcGVHDNoveltherapeuticstrategiesofcGVHDCIBMTR:GVHD发病率MatchedSibli

2、ngMatchedUnrelatedN=3158N=941AcuteGVHDGrade:II18%31%III-IV16%21%ChronicGVHD42%49%RingdénO,etal.Blood.2009;113:3110-3118.NIH新的GVHD分类标准(2005)AcuteGVHDclassicacuteGVHDlate-onsetacuteGVHDChronicGHVDClassicchronicGVHDOverlapsyndromeNIH分类标准最重要的变化是以临床表现和器官受累的程度，而不是移植后时间来进行分

3、类，这有利于临床医生作出更符合病理生理学改变的诊断和治疗策略FilipovichAH,etal.Biol.BloodMarrowTransplant.11(12),945–956(2005).GVHDclassificationaftertheNIHconsensusconferencePavleticSZ,andFowlerDHHematology2012;2012:251-264cGVHD发病的危险因素AcuteGVHDOlderageofrecipientanddonorFemalemultiparousdonorMism

4、atchedandunrelateddonorsPBSCproductDiseasetype:CML,Aplasticanemia↓HighCD34doseand/orT-celldoseSecondtransplantsDLIsCMV?影响cGVHD发病率的因素ClassificationProgressivepoorestprognosisQuiescentdenovo#1riskfactor:historyofacuteGVHDChangingriskfactorsOlderrecipientageDonors(unre

5、lated,haploidentic)Non-myeloablativeconditioningPeripheralbloodstemcellsourceDonorleukocyteinfusions(DLI)Leeetal.,BiolBloodMarrowTransplant2003;9:215-33.慢性GVHD的临床表现受累器官系统肯定为慢性GVHD的临床表现可能为慢性GVHD的临床表现皮肤硬皮病，扁平苔藓，白癜风，疤痕性脱发，毛发角化过度，皮肤挛缩，甲床发育异常湿疹样皮疹,皮肤干燥,斑丘疹,脱发,色素沉着黏膜扁平苔藓，口

6、干症，非感染性溃疡，角膜糜烂/非感染性结膜炎口腔干燥,干燥性角结膜炎消化道食管狭窄，脂肪泻食欲减退，吸收不良，体重减轻，腹泻，腹痛肝脏无特异性碱性磷酸酶升高,转氨酶增高，胆管炎，高胆红素血症泌尿生殖道阴道狭窄，扁平苔藓非感染性阴道炎，阴道萎缩肌肉骨骼/浆膜非特异性关节炎，肌炎，肌无力，浆膜炎，挛缩性关节固定关节痛血液系统无特异性血小板减少,嗜酸性细胞增多，自身免疫性血细胞减少肺闭塞性细支气管炎闭塞性细支气管炎伴机化性肺炎，间质性肺炎cGVHD:多形性的皮肤病变EpidermalcGVHDLichenplanus-likePapu

7、losquamousIchthyosiformPoikilodermaKeratosispilaris-likeAcralerythemaDermalcGVHDLichen-sclerosus-likeDermalsclerosisSubcutaneouscGVHDSubcutaneoussclerosisFasciitiscGVHDcGVHD：口腔黏膜溃疡TreisterNetal.Blood2012;120:3407-3418Pérez-SimónJAetal.Haematologica2012;97:1187-1195不同

8、类型cGVHD的预后MultivariateriskfactorprofilesacuteGVHDandchronicGVHDFlowersM,etal.Blood.2011;117(11):3214-3219)cGVHD危险度积分*Mild–nosignifi