Síndromes hipereosinofílicos. Mastocitosis sistémicas

Eosinophilia may be a reactive phenomenon mediated by cytokines (secondary) or may be a component of phenotype of an underlying hematologic malignancy (primary). Primary eosinophilia is classified as an idiopathic clonal disease depending on the presence or absence of certain molecular, cytogenetic or histological findings. Hypereosinophilic syndrome (HES) encompass a spectrum of clonal (primary SHE) or idiopathic (idiopathic SHE) diseases that have increased eosinophils in peripheral blood and tissue damage. Mast cell disease, or mastocytosis, includes a variety of disorders that are characterized by clonal proliferations of mast cells in one or multiple organs. The mastocytosis range from indolent and isolated proliferations to aggressive and systemic disorders. Mastocytosis is included in the myeloproliferative neoplasms category in the 2008 World Health Organization (WHO) classification. This chapter describes both disorders, recent classifications, clinical manifestations, systematic diagnosis and current treatments..