Groh Matthieu, Lefèvre Guillaume, Ackermann Félix, Étienne Nicolas, Kahn Jean-Emmanuel
Centre de référence des syndromes hyperéosinophiliques (CEREO). Service de médecine interne, hôpital Foch, Suresnes, France.
Centre de référence des syndromes hyperéosinophiliques (CEREO). Service de médecine interne et d'immunologie clinique, CHRU de Lille, Lille, France.
Rev Prat. 2019 Sep;69(7):767-773.
Hypereosinophilic syndromes. Hypereosinophilic syndromes (HES) is a protean condition defined by chronic blood eosinophilia ≥ 1.5 G/L (> 1 month) leading to eosinophilic-related organ damage. HES subtypes includes neoplastic (clonal) disorders (HESN, that comprises FIP1L1-PDGFRA- related chronic eosinophilic leukemia and myeloproliferative and myelodysplastic syndromes associated with eosinophilia) and reactive HES (HESR, that aggregates all conditions e.g. parasitic infections, adverse drug reactions, inflammatory or neoplastic diseases that lead to the production of Th2-related cytokines and thereby to non-clonal hypereosinophilia). HESR also includes the lymphoid variant of HES (HESL), a chronic clonal indolent T-cell lymphoproliferative disorder in which mature peripheral T cells secrete high amounts of IL-5, leading to the polyclonal expansion of eosinophils. Despite an extensive etiological workup, approximately 50% of HES remain of undetermined cause. HES-related clinical manifestations are highly diverse, but dermatological, respiratory and gastro-intestinal symptoms are the most frequent. The long-term prognosis is driven by cardiac involvement and, for patients with HESN and HESL, by the risk of acute transformation into high-grade hematological malignancies. Treatment of HESN relies on tyrosine kinase inhibitors (e.g. imatinib mesylate), while oral glucocorticoids are the usual the fist-line therapy for HESR (including SHEL). In this setting, second-line treatments include hydroxyurea and Peg-interferon alfa-2a. IL-5-targeted therapies are very promising (except for HESN). Yet, to date, their use is restricted to clinical trials and to a compassionate use program dedicated to severe and refractory patients.
高嗜酸性粒细胞综合征。高嗜酸性粒细胞综合征（HES）是一种具有多种表现的病症，其定义为慢性血液嗜酸性粒细胞增多≥1.5 G/L（持续>1个月）并导致嗜酸性粒细胞相关器官损害。HES亚型包括肿瘤性（克隆性）疾病（HESN，包括FIP1L1-PDGFRA相关慢性嗜酸性粒细胞白血病以及与嗜酸性粒细胞增多相关的骨髓增殖性和骨髓增生异常综合征）和反应性HES（HESR，涵盖所有导致产生Th2相关细胞因子从而引起非克隆性嗜酸性粒细胞增多的病症，如寄生虫感染、药物不良反应、炎症性或肿瘤性疾病）。HESR还包括HES的淋巴样变体（HESL），这是一种慢性克隆性惰性T细胞淋巴增殖性疾病，其中成熟外周T细胞分泌大量IL-5，导致嗜酸性粒细胞多克隆扩增。尽管进行了广泛的病因学检查，仍约50%的HES病因不明。HES相关的临床表现高度多样，但皮肤、呼吸和胃肠道症状最为常见。长期预后取决于心脏受累情况，对于HESN和HESL患者，还取决于急性转化为高级别血液系统恶性肿瘤的风险。HESN的治疗依赖于酪氨酸激酶抑制剂（如甲磺酸伊马替尼），而口服糖皮质激素通常是HESR（包括SHEL）的一线治疗方法。在这种情况下，二线治疗包括羟基脲和聚乙二醇干扰素α-2a。靶向IL-5的疗法非常有前景（HESN除外）。然而，迄今为止，它们的使用仅限于临床试验以及针对重症和难治性患者的同情用药项目。
