특발성 과다 호산구증후군의 치료에 대한 분석
Analysis on Treatment of the Idiopathic Hypereosinophilic Syndrome ; A Retrospective Long-Term Observation Study
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초록
The idiopathic hypereosinophilic syndrome is a very rare hematologic disorder characterized by persistent blood eosinophilia (more than 1,500/mm3) for more than six months in the absence of known causes and multiorgan dysfunction by eosinophil-related damage. Treatment of idiopathic hypereosinphilic syndrome is aimed at lowering the eosinophil count and improving the symptoms produced by eosinophilic end-organ damage. Steroid is the first line of therapy and can reduce end organ damage. Hydroxyurea and interferon-alpha can be used for steroidresistant patients. Steroid has been used with satisfactory results in the idiopathic hypereosinophilic syndrome,; however, the most appropriate initial dose and the duration of steroid therapy have not been studied. A retrospective study was performed to evaluate the treatment and response of patients with idiopathic hypereosinophilic syndrome during the study period of 60 months. A total of 9 patients, who were diagnosed as idiopathic hypereosinphilic syndrome at a single center, were reviewed. Seven patients were treated with steroids, 2 (28.6%) achieved complete response and 5 (71.4%) partial response, 1 month after the initiation of therapy. The median maximal daily dose of steroid was 27 mg (range 10~40mg). Two patients were treated with hydroxyurea 1,000 mg, and 2 (100%) achieved partial response 1 month after the treatment. In summary, although this study has limitations due to its retrospective design and small number, low dose steroid was an effective treatment for patients with idiopathic hypereosinophilic syndrome.
Abstract
The idiopathic hypereosinophilic syndrome is a very rare hematologic disorder characterized by persistent blood eosinophilia (more than 1,500/mm3) for more than six months in the absence of known causes and multiorgan dysfunction by eosinophil-related damage. Treatment of idiopathic hypereosinphilic syndrome is aimed at lowering the eosinophil count and improving the symptoms produced by eosinophilic end-organ damage. Steroid is the first line of therapy and can reduce end organ damage. Hydroxyurea and interferon-alpha can be used for steroidresistant patients. Steroid has been used with satisfactory results in the idiopathic hypereosinophilic syndrome,; however, the most appropriate initial dose and the duration of steroid therapy have not been studied. A retrospective study was performed to evaluate the treatment and response of patients with idiopathic hypereosinophilic syndrome during the study period of 60 months. A total of 9 patients, who were diagnosed as idiopathic hypereosinphilic syndrome at a single center, were reviewed. Seven patients were treated with steroids, 2 (28.6%) achieved complete response and 5 (71.4%) partial response, 1 month after the initiation of therapy. The median maximal daily dose of steroid was 27 mg (range 10~40mg). Two patients were treated with hydroxyurea 1,000 mg, and 2 (100%) achieved partial response 1 month after the treatment. In summary, although this study has limitations due to its retrospective design and small number, low dose steroid was an effective treatment for patients with idiopathic hypereosinophilic syndrome.
참고문헌 (15)
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