Chapter 15

Acute Myeloproliferative Disease

Eduardo Magalhaes Rego and Mariana Tereza de Lira Benicio

Abstract

Acute myeloproliferative disease is a heterogeneous group of malignant disorders. In spite of the great variability regarding genetic and clinical aspects, all forms present common mechanisms underlying their pathogenesis: the disruption of genes tightly involved in the control of cell differentiation, proliferation and/or apoptosis. Major progress has been made to better understand such events and recurrent genetic abnormalities have been acknowledged based on both physiopathologic and prognostic relevance: t(15;17), t(8;21), inv(16)/t(16;16), in addition to mutations in the genes NPM1, FLT3 and CEBPA. These genetic alterations have identified particular subgroups of patients and their resultant aberrant proteins have become targets for drug development, intending to improve therapy efficacy and to diminish its toxicity.

Total Pages: 201-217 (17)

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