특발성 과다 호산구증후군의 치료에 대한 분석 = Analysis on Treatment of the Idiopathic Hypereosinophilic Syndrome ; A Retrospective Long-Term Observation Study

다국어 초록 (Multilingual 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 (Reference)

1 Gotlib J, "World Health Organizationdefined eosinophilic disorders: 2011 update on diagnosis, risk stratification, and management" 86 : 677-688, 2011

2 Swerdlow S.H, "World Health Organization Classificationof Tumours of Haematopoietic andLymphoid Tissues" IARCPress 2008

3 Wardlaw A, "Williams hematology, 7th ed, In Williams hematology, 7th ed" Mc Graw-Hill 863-878, 2006

4 Gleich G.J, "Treatment of hypereosinophilic syndrome with imatinib mesilate" 359 : 1577-1578, 2002

5 Chusid D.C, "The hypereosinophilic syndrome, Analysis of fourteen cases with review of the literature" 54 : 1-27, 1975

6 Ault P, "Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate" 26 : 881-884, 2002

7 Schaller J.L, "Rapid and complete control of idiopathic hypereosinophilia with imatinib mesylate" 3 : 9-, 2001

8 Bain B.J, "Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1, In World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues" IARC Press 68-73, 2008

9 Kahn J.E, "Hypereosinophilic syndromes" 22 : 863-882, 2008

10 Ogbogu P.U, "Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy" 124 : 1319-1325, 2009

11 Brito B.F, "Clonal eosinophilic disorders and the hypereosinophilic syndrome" 1 : 129-145, 1997

12 Bain B.J, "Chronic eosinophilic leukaemia, not otherwise specified, In World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues" IARC Press 51-53, 2008

13 Klion A.D, "Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report" 117 : 1292-1302, 2006

14 Cools J, "A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome" 348 (348): 1201-1214, 2003

15 Chung J.P, "A Clinical Study on Eosinophilia ; With a Report of 5 Cases of Hypereosinophilic Syndrome" 23 (23): 127-137, 1988