HES及克隆性嗜酸性粒细胞增多症.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 Tefferi A,Gotlib J,Pardanani A.Hypereosinophilic syndrome and clonal eosinophilia:point-of-care diagnostic algorithm and treatment update.Mayo Clin Proc.2010;85(2):158-64.Acquired eosinophilia Acquired eosinophilia is operationally categorized into secondary,clonal,and idiopathic types.Causes of secondary eosinophilia include parasite infections,allergic or vasculitis conditions,drugs,and lymphoma.Clonal eosinophilia is distinguished from idiopathic eosinophilia by the presence of histologic,cytogenetic,or molecular evidence of an underlying myeloid malignancy.获得性嗜酸性粒细胞增多症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。Clonal eosinophilia The World Health Organization classification system for hematologic malignancies recognizes 2 distinct subcategories of clonal eosinophilia:chronic eosinophilic leukemia,not otherwise specified (CEL-NOC)and myeloid/lymphoid neoplasms with eosinophilia and mutations involving platelet-derived growth factor receptor alpha/beta(PDGFRA or PDGFRB)or fibroblast growth factor receptor 1(FGFR1).克隆性嗜酸性粒细胞增多症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。Clonal eosinophilia Clonal eosinophilia might also accompany other World Health Organization-defined myeloid malignancies,including chronic myelogenous leukemia,myelodysplastic syndromes,chronic myelomonocytic leukemia,and systemic mastocytosis.克隆性嗜酸性粒细胞增多症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。Hypereosinophilic syndrome(HES)Hypereosinophilic syndrome(HES),a subcategory of idiopathic eosinophilia,is defined by the presence of a peripheral blood eosinophil count of 1.5 x 10(9)/L or greater for at least 6 months(a shorter duration is acceptable in the presence of symptoms that require eosinophil-lowering therapy),exclusion of both secondary and clonal eosinophilia,evidence of organ involvement,and absence of phenotypically abnormal and/or clonal T lymphocytes.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1.5 x 10(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。Lymphocytic variant hyper eosinophilia The presence of the latter defines lymphocytic variant hyper eosinophilia,which is best classified under secondary eosinophilia.In the current review,we provide a simplified algorithm for distinguishing the various causes of clonal and idiopathic eosinophilia and discuss current therapy,including new drugs(imatinib mesylate,alemtuzumab,and mepolizumab).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.Diagnostic algorithm for clonal or idiopathic eosinophilia.CEL-NOS=chronic eosinophilic leukemia,not otherwise specified;FISH=fluorescence in situ hybridization;HES=hypereosinophilic syndrome;PDGFR=platelet-derived growth factor receptor;RT-PCR=reverse transcription polymerase chain reaction;TCR=T-cell receptor;WHO=World Health Organization.Tefferi A.Blood eosinophilia:a new paradigm in disease classification,diagnosis,and treatment.Mayo Clin Proc.2005;80(1):75-83.Tefferi A.Modern diagnosis and treatment of primary eosinophilia.Acta Haematol.2005;114(1):52-60.Tefferi A,Patnaik MM,Pardanani A.Eosinophilia:secondary,clonal and idiopathic.Br J Haematol.2006;133(5):468-92.Pardanani A,Tefferi A.Primary eosinophilic disorders:a concise review.Curr Hematol Malig Rep.2008;3(1):37-43.Tefferi A,Vardiman JW.Classification and diagnosis of myeloproliferative neoplasms:the 2008 World Health Organization criteria and point-of-care diagnostic algorithms.Leukemia.2008;22(1):14-22.Tefferi A,Gotlib J,Pardanani A.Hypereosinophilic syndrome and clonal eosinophilia:point-of-care diagnostic algorithm and treatment update.Mayo Clin Proc.2010;85(2):158-64.Corresponding author Ayalew Tefferi,MD Divisions of Hematology and Internal Medicine,Mayo Clinic,200 First Street SW,Rochester,Minn.,USA