Síndromes mieloproliferativosMyeloproliferative syndromes

The myeloproliferative syndromes are clonal disorders of the hematopoietic stem cell, leading to an abnormal proliferation of one or more myeloid cellular lineages. The clinical manifestations derive from the myeloproliferation and tipically includes splenomegaly and evolution to acute leukemia. In the year 2008 the WHO revised these syndromes and included them in the myeloproliferative category, highligting their clonal condition and integrating the new molecular data. The tyrosinkinase inhibitors have established a new paradigm in cancer treatment, approaching the chronic myeloid leukemia to the cure. The aim of the treatment in Policythemia vera and Essential thrombocythemia is to prevent vascular events. In Primary Myelofibrosis the treatment remains palliative and supportive. The recent advances in the understanding of the molecular pathogenesis with the discovery of the JAK2 mutations will bring new treatments in the next future