hypereosinophilic syndrome

Idiopathic hypereosinophilic syndrome (IHES) is a leukoproliferative disorder and refers to a situation when there is an unexplained prolonged eosinophilia with associated organ system dysfunction. The condition can affect several organ systems which includes:

  • heart: cardiac involvement in idiopathic hypereosinophilic syndrome
  • lung: pulmonary involvement in idiopathic hypereosinophilic syndrome
    • can occur in up to 40% of cases
    • pulmonary manifestations are sometimes categorized under the spectrum of eosinophilic lung disease 
  • central nervous system: CNS involvement in idiopathic hypereosinophilic syndrome
  • peripheral nervous system: peripheral nervous system involvement in idiopathic hypereosinophilic syndrome
  • eyes: ocular involvement in idiopathic hypereosinophilic syndrome
  • joints: rarely seen rheumatological manifastations of idiopathic hypereosinophilic syndrome

Pathology

Individuals have prolonged raised total leukocyte counts with raised eosinophil counts. Usually the total leukocyte counts are less than 25,000/μL with 30-70% comprising of eosinophils. At times extremely high leukocyte counts  (>90,000/μL) can also develop which can then result in a poorer prognosis.

Radiographic features

Radiographic features are probably best discussed under individual categories.

History and etymology

It was first described by W R Hardy and R E Anderson in 1968 .

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