New research progress in pathogenesis and therapy of myeloproliferative neoplasms

Abstract

Classical breakpoint cluster region/Abelson leukemia virus (BCR-ABL) fusion gene is negative in myeloproliferative neoplasms (MPN), including polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis(PMF), ect.. These kind of diseases are caused by abnormal clonal proliferation of hematopoietic stem cells, and there are high frequency of Janus kinase (JAK)2 V617F, JAK2 gene exon 12 mutations, thrombopoietin receptor MPL W515L/K, calreticulin (CALR) mutation, etc.. The relationships between these genetic changes and the pathogenesis of MPN are not fully clear. In 2008, the World Health Organization (WHO) diagnostic criteria for MPN has been revised, and JAK2 gene mutations has been included. Drug treatment for MPN includes α interferon and/or hydroxyurea and thalidomide. In recent years, some new drugs, especially targeted agents are very promising, and have been used in the clinical treatment of MPN. In order to guide diagnosis and treatment of MPN effectively, this article reviews literatures on progress of pathogenesis and treatment in MPN. Key words: Myeloproliferative disorders; Janus kinase 2; MPL protein, human; Calreticulin; Drug therapy