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Hypereosinophilic Syndrome (HES) / Chronic Eosinophilic Leukaemia (CEL)
How is HES/CEL diagnosed?
Tests to diagnose CEL include a complete blood count and a medical examination to determine whether there is organ damage.
There are three requirements for a diagnosis of CEL:
- Eosinophil counts are elevated (more than 1500 per microlitre) for at least 6 months
- Causes such as infection or allergy have been ruled out
- There is evidence of organ damage
What are the prospects for people with HES/CEL?
Prospects for people with CEL vary from person to person. It depends on how severe the disease is, which bodily organs are affected, and the success of treatment. In some cases, CEL may be fatal.
Can CEL be eliminated from the body?
CEL cannot be eliminated, but it can often be treated.
How is HES/CEL treated?
Treatment of CEL depends on which bodily organs are involved and how severe the disease is. The most common treatment for CEL consists of high doses of steroids (medications in the cortisone family). Other treatments include medications that suppress the immune system, and anticancer therapies.
Under normal circumstances, our body regulates the production of white blood cells very precisely by means of a targeted activation of tyrosine kinases, which start this production. But sometimes defects in the DNA cause these tyrosine kinases to be active continuously, giving rise to diseases like leukemia. HES/CEL are both caused by this kind of defective activation of the tyrosine kinase FIP1-like-1–platelet-derived growth factor receptor-α (FIP1L1-PDGFRα). (Gotlib et al, 2004, p1a)
New treatments for HES are now being studied in clinical trials, including one that targets the kinase protein.
New references
- Gotlib J et al. The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management Blood 2004;103:2879-2891.
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