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Hypereosinophilic Syndrome
Treatment
Corticosteroids are the mainstay of treatment for HES, although the results with these and other agents have been generally disappointing.1,2 Lack of steroid responsiveness suggests the need for cytotoxic therapy with options such as vincristine, etoposide, and alkylating agents.3,4 Interferon-alpha has proven to be useful in some patients, although adverse events are a dose-limiting problem.1
Course
The course of HES is highly variable, ranging from minimal disease with long survival probability to rapid mortality due to sudden, severe heart failure or acute leukemia.5,6
Disease subtype has important implications with respect to predicting treatment response, end-organ manifestations, and prognosis.1 The myeloproliferative type of HES is characterized by unresponsiveness to corticosteroid therapy and a particularly poor prognosis: Despite the treatments described above, mortality among patients with myeloproliferative HES exceeds 50%.1 Lymphoproliferative HES is less frequently fatal, but is associated with both substantial morbidity and a high risk of progression to T-cell lymphoma.1
Emerging Therapies
Several therapeutic approaches are under investigation that utilize a molecular-targeted approach to the treatment of HES.1
One such approach involves use of tyrosine kinase inhibitors that target the fusion protein that transforms eosinophilic cells or their stem cells in patients with FIP1L1-PDGFRA-positive disease. This approach shows considerable promise in the management of patients with HES. Clinical trials are underway.
Clinical Trials
For U.S. Residents Only
References:
- Klion AD. Recent advances in the diagnosis and treatment of hypereosinophilic syndromes. Hematology (Am Soc Hematol Educ Program). 2005;209-214.
- Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54:1-27.
- Gotlib J, Cools J, Malone JM 3rd, et al. The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management. Blood. 2004;103:2879-2891.
- Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by the fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003;348:1201-1214.
- Roufosse F, Cogan E, Goldman. The hypereosinophilic syndrome revisited. Annu Rev Med. 2003;54:169-184.
- Anghel G, De Rosa L, Ruscio C, et al. Efficacy of imatinib mesylate in a patient with idiopathic hypereosinophilic syndrome and severe heart involvement. Tumori. 2005;91:67-70.
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