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Hypereosinophilic syndromes: Treatment

Florence Roufosse, MD
Amy D Klion, MD
Peter F Weller, MD, FACP
Section Editor
Bruce S Bochner, MD
Deputy Editors
Anna M Feldweg, MD
Alan G Rosmarin, MD


The hypereosinophilic syndromes (HES) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. The decision to treat is based upon the patient's presentation as well as laboratory findings and the results of mutational analysis. HES may develop in the setting of common underlying causes of hypereosinophilia (HE), such as parasitic infections, drug allergy, and neoplastic disease (ie, paraneoplastic HE). In such cases, treatment of the underlying condition is generally sufficient to reverse complications of HE, although short-term glucocorticoids may be necessary to accelerate resolution. However, there are other situations in which the consequences of eosinophil-mediated organ damage merit immediate anti-eosinophil therapies, apart from simply treating the underlying disease. Patients in urgent need of intervention include those with myeloproliferative HES (M-HES), particularly those with mutations resulting in the fusion of the genes for Fip1-like1 (FIP1L1) and platelet-derived growth factor receptor alpha (PDGFRA). These patients should be treated without delay whether they have an aggressive course with disabling complications or not, both to prevent irreversible damage (eg, to heart or central nervous system) that may develop rapidly in some cases and because of high mortality in the absence of treatment.

The treatment and prognosis of HES will be reviewed here. The clinical features, pathophysiology, evaluation, and diagnosis of HES are discussed separately. (See "Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis".)


Some patients with hypereosinophilic syndromes (HES) require immediate treatment, while others can be safely monitored. The urgency with which patients are treated depends upon the severity of hypereosinophilia (HE) and the presence of signs and symptoms of the disease [1]:

In rare instances, patients may present with extremely elevated eosinophil levels (eg, absolute eosinophil count [AEC] above 100 x 109/L [>100,000 cells/microL]) or signs and symptoms of hyperleukocytosis. In these situations, hypercellularity should be rapidly reduced before additional treatments are considered. (See 'Immediate treatment for severe disease' below.)

The majority of patients are symptomatic and have less severe degrees of eosinophilia. The goals of pharmacologic therapy are to reduce the signs and symptoms of eosinophilic disease and to maintain the AEC below 1.5 x 109/L (1500 cells/microL) to help prevent the development of end-organ damage.


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Literature review current through: Sep 2016. | This topic last updated: Nov 6, 2015.
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