单中心60例高嗜酸粒细胞综合征的临床特征及长期疗效  

Clinical characteristics and long-term therapeutic effects of 60 patients with idiopathic hypereosinophilic syndrome in a single center

作　　者：曲士强[1] 秦铁军[1] 徐泽锋 张悦 艾小菲 李冰 张宏丽 方力维 潘丽娟 胡耐博 肖志坚 

机构地区：[1]中国医学科学院、北京协和医学院血液学研究所、血液病医院,实验血液学国家重点实验室,天津300020

出　　处：《中华血液学杂志》2016年第10期881-885,共5页Chinese Journal of Hematology

基　　金：国家自然科学基金（81530008、81370611、81270585、81470297）;天津市自然科学基金（16JCQNJC11400）;协和学者与创新团队发展计划

摘　　要：目的分析高嗜酸粒细胞综合征（HES）的临床特征和长期疗效。方法回顾性分析接受糖皮质激素单药治疗的60例初诊HES患者的临床资料，通过随访获得患者生存情况及死亡原因。结果全部60例HES患者中，男45例，女15例，中位年龄38（11-80）岁。常见受累器官依次为皮肤（55.0%）、胃肠道（40.0%）、肺（35.0%）、心脏（13.3%）、血管（10.0%）和神经肌肉（10.0%）。单器官受累27例（45.0%），2个器官受累22例（36.7%），2个以上器官受累11例（18.3%）。糖皮质激素中位诱导治疗剂量相当于泼尼松30（15-60）mg/d，治疗总有效率[完全缓解（CR）＋部分缓解（PR）]为88.3%（53/60）。联合或更换其他药物后总有效率提升至93.3%（56/60）。38例治疗有效患者至随访截止时仍接受糖皮质激素单药（33例）或联合（5例）维持治疗，糖皮质激素的中位治疗时间为51（5-92）个月，中位维持剂量相当于泼尼松5（1.25-40）mg/d。20例患者因疾病缓解后依从性差或治疗无效停用糖皮质激素。HES患者5年总体生存率为（90.0±4.3）%，主要死亡原因为心脏功能异常。结论HES对糖皮质激素治疗敏感，多数疗效欠佳的患者可以通过联合用药改善疗效。心脏功能异常是患者的主要死亡原因。ObjectiveTo analyze the long term outcome of patients with hypereosinophilic syndrome（HES）.MethodsThe clinical characteristics and efficacy of 60 newly diagnosed HES patients who received corticosteroids（CS）monotherapy were retrospectively analyzed. The survival and death causes of patients were obtained by follow-up.ResultsOf all 60 HES patients, 45 were male and 15 female. The median age was 38（11-80）years old. The most frequent organ involvement of HES occurred in cutaneous（55.0%）, gastrointestinal（40.0%）, pulmonary（35.0%）, cardiac（13.3%）, vascular（10.0%）and neuromuscular system（10.0%）. Single organ involvement was observed in 45.0% of the patients, two or at least three organ involvements were observed in 36.7% and 18.3%, respectively. The median daily dose of prednisone equivalent was 30（15-60）mg. The total response rate（CR plus PR）was 88.3%, and the rate was elevated to 93.3% after receiving alterative or combined treatment regimens. Thirty-eight patients with response to treatment received corticosteroid（CS）as mono（33 cases）or combined（5 cases）maintenance treatment with a median duration of 51（5-92）months; the median maintenance daily dose of prednisone equivalent was 5（1.25-40）mg. Twenty patients experienced cessation of CS. The main causes of patients＇ withdrawal were poor compliance after CR or ineffective treatment. The 5-year overall survival was（90.0±4.3）%, and the main cause of mortality was cardiac dysfunction.ConclusionCS was highly effective on HES with manageable side effects. Most patients who have not obtained satisfactory effect could improve response via combination therapy. Cardiac dysfunction was the most common cause of mortality.

关 键 词：嗜酸细胞增多综合征 糖皮质激素 疾病特征 治疗结果 

分 类 号：R55[医药卫生—血液循环系统疾病]