Eosinophilic myeloid disorders

The discovery of therapeutically relevant mutations involving platelet-derived growth factor receptors alpha and beta (PDGFRA and PDGFRB) changed the way we evaluate and treat patients with clonal eosinophilia. Despite our improved understanding of the pathobiology of clonal eosinophilia, more than 50% of patients are diagnosed with idiopathic disease, 10% to 20% with a clonal myeloid disorder, and the remainder with a lymphocytic variant. The World Health Organization classification of tumors recognized the importance of a semi-molecular classification of eosinophilc myeloid disorders and divided them into two major subgroups: (1) myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or fibroblast growth factor receptor 1 (FGFR1); and (2) chronic eosinophilic leukemia, not otherwise specified. A key challenge remains the identification of tyrosine kinase responsive molecular lesions in patients in whom the pathogenesis of clonal eosinophilia remains unclear.