Bockenstedt P L, Santinga J T, Bolling S F
Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor 48109-0724.
Am J Hematol. 1994 Mar;45(3):248-51. doi: 10.1002/ajh.2830450311.
Idiopathic hypereosinophilic syndrome (HES) is a poorly understood disorder characterized by a markedly elevated peripheral blood eosinophil count in the absence of known associated causes of hypereosinophilia. Idiopathic hypereosinophilic syndrome is associated with eosinophil-induced organ damage, including endomyocardial and pulmonary fibrosis, stroke, and gastrointestinal disease. Treatment of idiopathic HES is centered on the reduction of peripheral circulating eosinophils in an effort to diminish tissue infiltration and destruction. Multiple cytotoxic agents have been tried, with variable results. Prednisone and hydroxyurea have remained the therapies of choice in long term treatment of idiopathic HES. We report here the successful 2 year treatment of aggressive idiopathic HES, refractory to hydroxyurea and prednisone, with alpha-interferon.
特发性高嗜酸性粒细胞综合征（HES）是一种了解甚少的疾病，其特征是外周血嗜酸性粒细胞计数显著升高，且不存在已知的高嗜酸性粒细胞血症相关病因。特发性高嗜酸性粒细胞综合征与嗜酸性粒细胞诱导的器官损害有关，包括心内膜和肺纤维化、中风及胃肠道疾病。特发性HES的治疗以减少外周循环嗜酸性粒细胞为核心，以努力减少组织浸润和破坏。已尝试多种细胞毒性药物，结果各异。泼尼松和羟基脲一直是特发性HES长期治疗的首选疗法。我们在此报告用α干扰素成功治疗了2年的侵袭性特发性HES，该患者对羟基脲和泼尼松耐药。
