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Hypereosinophilic Syndrome
Pathophysiology
The basic pathology of HES is the accumulation of eosinophils in organ tissues or systems. The pathogenesis of the disorder is poorly defined. However, recent evidence points to etiologically distinct myeloproliferative- and lymphoproliferative major subtypes of the disorder.1
Myeloproliferative HES is associated with a novel fusion tyrosine kinase—FIP1L1-PDGFRA—that is a consequence of an interstitial chromosomal deletion.1,2 Up to 50% of patients with HES may have this PDGFRA-associated disease.1 In patients with the lymphoproliferative variant of HES, eosinophil overproduction appears to result from the production of eosinophilopoietic cytokines (particularly interleukin 5) by clonal populations of abnormal activated T lymphocytes.1
References:
- Klion AD. Recent advances in the diagnosis and treatment of hypereosinophilic syndromes. Hematology (Am Soc Hematol Educ Program). 2005;209-214.
- Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by the fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003;348:1201-1214.
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