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

Objective To analyze the long term outcome of patients with hypereosinophilic syndrome(HES). Methods The 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. Results Of 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. Conclusion CS 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. Key words: Hypereosinophilic syndrome; Glucocorticoids; Disease attributes; Treatment outcome