Hypereosinophilic syndrome diagnostic criteria and variants
HES are disorders marked by sustained over production of
eosinophils with damage to multiple organs due to eosinophil infiltration and
mediator protease. Cardiac involvement is present in 40% of cases.
For the diagnosis of HES the following criteria need to be met:
- Blood eosinophilia is >1500microliter for >6 months
- No other etiologies for eosinophilia like parasitic or allergic disorders
- Signs and symptoms of end organ dysfunction
There are 3 variants of HES
Variants with clonal abnormalities of
- Eosinophil lineage
- T lymphocytic variants,
- Myeloproliferative variant of HES.
Blast transformation could occur after many years.
True idiopathic hypereosinophilic syndrome is generally indolent;
however, patients with characteristics that are suggestive of a myeloproliferative/neoplastic
disorder and those who manifest CHF have a worse prognosis.
