World Health Organization-defined eosinophilic disorders: 2011 update on diagnosis, risk stratification, and management
World Health Organization-defined eosinophilic disorders: 2011 update on diagnosis, risk stratification, and management
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DOI：
10.1002/ajh.22062
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发表时间：
2011-08-01
影响因子：
12.8
通讯作者：
Gotlib, Jason
中科院分区：
文献类型：
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作者：
Gotlib, Jason
疾病概述：嗜酸性粒细胞病包括广泛的非血液学（继发性或反应性）和血液学（原发性，克隆性）疾病，具有潜在的终末器官损伤。诊断：嗜酸性粒细胞增多症通常被定义为外周血嗜酸性粒细胞计数大于1500 /mm(3)，并可能与组织损伤有关。在排除嗜酸性粒细胞增多症的继发性原因后，原发性嗜酸性粒细胞增多症的诊断评估依赖于血液和骨髓的形态学检查、标准细胞遗传学、荧光原位杂交、流式免疫细胞术和t细胞克隆性评估的结合，以检测急性或慢性骨髓或淋巴细胞增生性疾病的组织病理学或克隆证据。风险分层：疾病预后依赖于嗜酸性粒细胞增多症亚型的确定。在对嗜酸性粒细胞增多症的继发原因进行评估后，2008年世界卫生组织建立了疾病亚型的半分子分类方案，包括“嗜酸性粒细胞增多症和PDGFRA、PDGFRB或FGFR1异常的髓系和淋巴系肿瘤”、“慢性嗜酸性粒细胞白血病，未另行指定”（CEL、NOS）、淋巴细胞变异性嗜酸性粒细胞增多症和特发性嗜酸性粒细胞增多综合征（HES），这是一种排除诊断。风险适应疗法：治疗的目标是减轻嗜酸性粒细胞介导的器官损伤。对于嗜酸性粒细胞较轻的患者（例如< 1500 /mm），没有器官受损伤的症状或体征，可以采取密切随访的观察和等待方法。鉴定重排PDGFRA或PDGFRB是至关重要的，因为这些疾病对伊马替尼的敏感性很高。皮质类固醇是淋巴细胞变异性嗜酸性粒细胞增多症和HES患者的一线治疗。羟基脲和干扰素作为HES的初始治疗和类固醇难治性病例已被证明有效。除羟基脲外，二线细胞毒性化疗药物和造血细胞移植已被用于侵袭性HES和CEL，报道的结果仅限于有限数量的患者。尽管抗IL-5 （mepolizumab）和抗cd52 （alemtuzumab）抗体已经进行了临床试验，但它们在原发性嗜酸性粒细胞疾病和HES中的治疗利基尚未建立。点。中华血液学杂志，2011,36(6):678-688。(C) 2011 Wiley-Liss, Inc。
Disease overview: The eosinophilias encompass a broad range of non-hematologic (secondary or reactive) and hematologic (primary, clonal) disorders with potential for end-organ damage.Diagnosis: Hypereosinophilia has generally been defined as a peripheral blood eosinophil count greater than 1,500/mm(3) and may be associated with tissue damage. After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on a combination of morphologic review of the blood and marrow, standard cytogenetics, fluorescent in situ-hybridization, flow immunocytometry, and T-cell clonality assessment to detect histopathologic or clonal evidence for an acute or chronic myeloid or lymphoproliferative disorder.Risk stratification: Disease prognosis relies on identifying the subtype of eosinophilia. After evaluation of secondary causes of eosinophilia, the 2008 World Health Organization establishes a semi-molecular classification scheme of disease subtypes including 'myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1', 'chronic eosinophilic leukemia, not otherwise specified' (CEL, NOS), lymphocyte-variant hypereosinophilia, and idiopathic hypereosinophilic syndrome (HES), which is a diagnosis of exclusion.Risk-adapted therapy: The goal of therapy is to mitigate eosinophil-mediated organ damage. For patients with milder forms of eosinophilia (e.g. < 1,500/mm(3)) without symptoms or signs of organ involvement, a watch and wait approach with close-follow-up may be undertaken. Identification of rearranged PDGFRA or PDGFRB is critical because of the exquisite responsiveness of these diseases to imatinib. Corticosteroids are first-line therapy for patients with lymphocyte-variant hypereosinophilia and HES. Hydroxyurea and interferon-alpha have demonstrated efficacy as initial treatment and steroid-refractory cases of HES. In addition to hydroxyurea, second line cytotoxic chemotherapy agents and hematopoietic cell transplant have been used for aggressive forms of HES and CEL with outcomes reported for limited numbers of patients. Although clinical trials have been performed with anti IL-5 (mepolizumab) and anti-CD52 (alemtuzumab) antibodies, their therapeutic niche in primary eosinophilic diseases and HES have yet to be established. Am. J. Hematol. 86:678-688, 2011. (C) 2011 Wiley-Liss, Inc.