Blood Eosinophilia: A New Paradigm in Disease Classification, Diagnosis, and Treatment

Tefferi, Ayalew
January 2005
Mayo Clinic Proceedings;Jan2005, Vol. 80 Issue 1, p75
Academic Journal
Acquired blood eosinophilia is considered either a primary or a secondary phenomenon. Causes of secondary (ie, reactive) eosinophilia include tissue-invasive parasitosis, ailergic or inflammatory conditions, and malignancies in which eosinophils are not considered part of the neoplastic process. Primary eosinophilia is classified operationally into 2 categories: clonal and Idiopathic. Clonal eosinophilia stipulates the presence of either cytogenetic evidence or bone marrow histological evidence of an otherwise classified hematologic malignancy such as acute leukemia or a chronic myeloid disorder, idiopathic eosinophilia is a diagnosis of exclusion (ie. not secondary or clonal). Hypereosinophilic syndrome is a subcategory of idiopathic eosinophilia: diagnosis requires documentation of both sustained eosinophilia (absolute eosinophil count ⩽1500 cells/μL for at least 6 months) and target organ damage (eg, involvement of the heart, lung, skin, or nerve tissue). Genetic mutations involving the platelet-derived growth factor receptor genes (PDGFR-α and PDGFR-β) have been pathogenetically linked to clonal eosinophilia, and their presence predicts treatment response to imatinib. Accordingly, cytogenetic and/or molecular investigations for the presence of an imatinibsensitive molecular target should accompany current evaluation for primary eosinophilia. In the absence of such a drug target, specific treatment is dictated by the underlying hematologic malignancy in cases of clonal eosinophilia; however, the initial treatment of choice for symptomatic patients with hypereosinophilic syndrome is prednisone and/or interferon alfa.


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