Onkológia 6/2012

New trends in diagnosis and treatment of essential thrombocythemia and myelofibrosis

Landmark discoveries occurring in the last few years have greatly advanced our knowledge of pathophysiology and contributed to a the reassessment of the diagnostic criteria of myeloproliferative neoplasms. On the other hand, the prognostic relevance of mutational abnormalities is still largely debated and very likely it is overall modest. Thus, the criteria employed for patients risk stratification are still exclusively based on clinical considerations. On the other hand, we are witnessing a remarkable number of ongoing clinical trials with drugs belonging to different classes that include interferon, inhibitors of histone deacethylases and JAK2 inhibitors, which hold the promise to improve the therapeutic approach to at least selected categories of patients with essential thrombocythemia and myelofibrosis. In this review, we will briefly present the current approach to the diagnosis and treatment of ET and MF aiming at illustrating, whenever possible, how novel molecular information resulted in modification of our clinical approach.

Keywords: essential thrombocythemia, myelofibrosis, risk factors, cytoreductive treatment, hydroxyurea, anagrelid, interferons, JAK2 inhibitors, new drugs.