HES及克隆性嗜酸性粒细胞增多症

高嗜酸性粒细胞综合症及嗜酸性粒细胞增多症：诊断要点和治疗进展TefferiA,GotlibJ,PardananiA.Hypereosinophilicsyndromeandclonaleosinophilia:point-of-carediagnosticalgorithmandtreatmentupdate.MayoClinProc.2010;85(2):158-64.Acquiredeosinophilia•Acquiredeosinophiliaisoperationallycategorizedintosecondary,clonal,andidiopathictypes.•Causesofsecondaryeosinophiliaincludeparasiteinfections,allergicorvasculitisconditions,drugs,andlymphoma.•Clonaleosinophiliaisdistinguishedfromidiopathiceosinophiliabythepresenceofhistologic,cytogenetic,ormolecularevidenceofanunderlyingmyeloidmalignancy.获得性嗜酸性粒细胞增多症•获得性嗜酸性粒细胞增多症被人为的分为继发性,克隆性和特发性三种类型。•继发性嗜酸性粒细胞增多的原因包括：寄生虫感染、过敏或血管炎,药物和淋巴瘤。•克隆性嗜酸性粒细胞增多是区别于特发性嗜酸性粒细胞增多的，通过组织学、细胞遗传学、或潜在的髓系恶性增生的分子证据。Clonaleosinophilia•TheWorldHealthOrganizationclassificationsystemforhematologicmalignanciesrecognizes2distinctsubcategoriesofclonaleosinophilia:chroniceosinophilicleukemia,nototherwisespecified(CEL-NOC)andmyeloid/lymphoidneoplasmswitheosinophiliaandmutationsinvolvingplatelet-derivedgrowthfactorreceptoralpha/beta(PDGFRAorPDGFRB)orfibroblastgrowthfactorreceptor1(FGFR1).克隆性嗜酸性粒细胞增多症•世界卫生组织恶性血液病分类系统将克隆性嗜酸性粒细胞增多症分为2个不同的亚型：未另行规定的慢性嗜酸性白血病和伴有血小板源生长因子受体α/β或纤维母细胞生长因子受体1基因突变的髓系/淋巴系肿瘤。Clonaleosinophilia•ClonaleosinophiliamightalsoaccompanyotherWorldHealthOrganization-definedmyeloidmalignancies,includingchronicmyelogenousleukemia,myelodysplasticsyndromes,chronicmyelomonocyticleukemia,andsystemicmastocytosis.克隆性嗜酸性粒细胞增多症•克隆性嗜酸性粒细胞增多症也可能伴发其他世界卫生组织定义的髓系恶性肿瘤,包括慢性粒细胞性白血病、骨髓增生异常综合征,慢性髓单核细胞白血病、系统性肥大细胞增多症。Hypereosinophilicsyndrome(HES)•Hypereosinophilicsyndrome(HES),asubcategoryofidiopathiceosinophilia,isdefinedbythepresenceofaperipheralbloodeosinophilcountof1.5x10(9)/Lorgreaterforatleast6months(ashorterdurationisacceptableinthepresenceofsymptomsthatrequireeosinophil-loweringtherapy),exclusionofbothsecondaryandclonaleosinophilia,evidenceoforganinvolvement,andabsenceofphenotypicallyabnormaland/orclonalTlymphocytes.高嗜酸性粒细胞综合征•高嗜酸性粒细胞综合征，即特发性嗜酸性粒细胞增多症的一个亚类,被定义为存在外周血嗜酸性粒细胞计数1.5x10(9)/L或更高至少6个月(当出现需要给予降低嗜酸性粒细胞治疗的症状时，更短时间内是可以接受的)，同时需要排除继发性和克隆性嗜酸性粒细胞增多，克隆性嗜酸性粒细胞增多的证据包括器官受累,没有表型上的异常和/或克隆T淋巴细胞增多症。Lymphocyticvarianthypereosinophilia•Thepresenceofthelatterdefineslymphocyticvarianthypereosinophilia,whichisbestclassifiedundersecondaryeosinophilia.•Inthecurrentreview,weprovideasimplifiedalgorithmfordistinguishingthevariouscausesofclonalandidiopathiceosinophiliaanddiscusscurrenttherapy,includingnewdrugs(imatinibmesylate,alemtuzumab,andmepolizumab).淋巴细胞变异型高嗜酸性粒细胞增多症•后者的存在定义为淋巴细胞变异型高嗜酸性粒细胞增多症,最好将其归类为继发性嗜酸性粒细胞增多症。•在当前的这篇评论性文章中,我们提供了一个简化的算法区别各种原因引起的克隆性和特发性嗜酸性粒细胞增多并讨论当前的治疗方法,包括新药(甲磺酸伊马替尼,CD52单抗-阿仑单抗和IL-5单抗-美泊利单抗)。FIGURE.Diagnosticalgorithmforclonaloridiopathiceosinophilia.CEL-NOS=chroniceosinophilicleukemia,nototherwisespecified;FISH=fluorescenceinsituhybridization;HES=hypereosinophilicsyndrome;PDGFR=platelet-derivedgrowthfactorreceptor;RT-PCR=reversetranscriptionpolymerasechainreaction;TCR=T-cellreceptor;WHO=WorldHealthOrganization.TefferiA.Bloodeosinophilia:anewparadigmindiseaseclassification,diagnosis,andtreatment.MayoClinProc.2005;80(1):75-83.TefferiA.Moderndiagnosisandtreatmentofprimaryeosinophilia.ActaHaematol.2005;114(1):52-60.TefferiA,PatnaikMM,PardananiA.Eosinophilia:secondary,clonalandidiopathic.BrJHaematol.2006;133(5):468-92.PardananiA,TefferiA.Primaryeosinophilicdisorders:aconcisereview.CurrHematolMaligRep.2008;3(1):37-43.TefferiA,VardimanJW.Classificationanddiagnosisofmyeloproliferativeneoplasms:the2008WorldHealthOrganizationcriteriaandpoint-of-carediagnosticalgorithms.Leukemia.2008;22(1):14-22.TefferiA,GotlibJ,PardananiA.Hypereosinophilicsyndromeandclonaleosinophilia:point-of-carediagnosticalgorithmandtreatmentupdate.MayoClinProc.2010;85(2):158-64.Correspondingauthor•AyalewTefferi,MD•DivisionsofHematologyandInternalMedicine,MayoClinic,200FirstStreetSW,Rochester,Minn.,USA