Chronic Myeloproliferative Diseases
Pp. 193-200 (8)
Katia B. Barbosa Pagnano
The Myeloproliferative Neoplasms (MPN) are clonal hematopoietic stem cell disorders
characterized by proliferation of one or more of the myeloid lineages, hipercellularity of the bone
marrow with hematopoietic maturation and increased numbers of granulocytes, red blood cells and/or
platelets in the peripheral blood. Several genes are involved in the pathogenesis of these diseases, like
bcr-abl in chronic myeloid leukemia, JAK-2 in polycithemia vera, myelofibrosis and essential
thrombocithemia, c-kit in systemic mastocytosis and PDGRF in chronic eosinophilic leukemia. The
advances in the understanding of the pathogenesis of the MPN has improved the diagnosis and will
contribute in the development of target therapies for these diseases.
BCR-ABL Breakpoints, major breakpoint cluster region (Mbcr ), Role of BCR-ABL, Bcr-Abl
signaling pathway, JAK2 signaling pathway, Chronic Myeloid Leukemia (CML), Polycythemia Vera,
Essential Thrombocithemia, Primary Myelofibrosis.
Centro de Hematologia e Hemoterapia da Unicamp, Rua Carlos Chagas 480, Campinas SP, Brazil CEP- 13083-970.