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Hypereosinophilic Syndrome

Kristen M. Wyrick, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Hypereosinophilic syndrome (HES): a group of disorders characterized by an overproduction of eosinophils that subsequently infiltrate and damage multiple organs

  • Hypereosinophilia (HE)...

DIAGNOSIS

  • A persistently elevated eosinophil count >1.5 × 109 cells/L on two examinations at least 1 month apart and/or tissue HE (2)[C]

  • Eosinophil-mediated organ damage in which other potential cau...

TREATMENT

  • Consider FIP1L1-PDGFRA (F/P) transcript status.

  • Initial treatment involves a trial of glucocorticoids alone in all patients except those with F/P+ (2)[B].

MEDICATION

  • F/P+HES

    • Tyrosine kinase inhi...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Depends on etiology of disease, response to treatment, and severity of end-organ damage 

Patient Monitoring

  • Weekly CBC on treatment; longer when stable

  • L-HES: increa...

REFERENCES

1
Pardanani A, Brockman SR, Paternoster SF, et al. FIP1L1-PDGFRA fusion: prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia. Blood. ...

ADDITIONAL READING

  • Chusid MJ, Dale DC, West BC, et al. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore).  1975;54(1):1–27.

  • Curtis C, O...

CODES

ICD10

D72.1 Eosinophilia 

ICD9

288.3 Eosinophilia 

SNOMED

423294001 idiopathic hypereosinophilic syndrome (disorder) 

CLINICAL PEARLS

  • HES encompasses a group of diseases with eosinophils >1,500/μL and end-organ damage without an identifiable secondary cause.

  • F/P+ transcript status should be determined to help guide...

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