HES及克隆性嗜酸性粒细胞增多症解读

Corresponding author
• Ayalew Tefferi , MD • Divisions of Hematology and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, Minn., USA
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.
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。
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.
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。
Tefferi A, Gotlib J, Pardanani A. Hypereosinophilic syndrome and clonal eosinophilia: pointof-care diagnostic algorithm and treatment update. Mayo Clin Proc. 2010;85(2):158-64.
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Байду номын сангаас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.
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).
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。
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.
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 plateletderived growth factor receptor alpha/beta (PDGFRA or PDGFRB) or fibroblast growth factor receptor 1 (FGFR1).
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)。
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.
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.
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.
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: pointof-care diagnostic algorithm and treatment update. Mayo Clin Proc. 2010;85(2):158-64.