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.
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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.
The condition can affect several organ systems which includes:
-
heart: cardiac involvement in idiopathic hypereosinophilic syndrome
- the heart can be a commonly involved organ
- has three stages and can cause
-
lung: pulmonary involvement in idiopathic hypereosinophilic syndrome
- can occur in up to 40% of cases 7.9
- pulmonary manifestations are sometimes categorized under the spectrum of eosinophilic lung disease 9
-
gastrointestinal system:
- 70% of those affected have history of food allergies or atopic disorders
- idiopathic eosinophilic esophagitis
- eosinophilic gastroenteritis
- eosinophilic colitis 10
- 70% of those affected have history of food allergies or atopic disorders
- central nervous system5: 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
Radiographic features
Radiographic features are probably best discussed under individual categories.
Ultrasound
Ultrasound may show hypoechoic lesions if there is liver involvement 11.
History and etymology
It was first described by W R Hardy and R E Anderson in 1968 4.
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