HES及克隆性嗜酸性粒细胞增多症(1)PPT课件.ppt

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 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 carediagnostical