Onkológia 6/2020

Diagnosis prefibrotic stage of myelofibrosis – reason for optimism and concern

The prefibrotic stage of myelofibrosis is now accepted as a separate subgroup of chronic myeloproliferative neoplasms. The latest classification of the World Health Organization from 2016 includes two stages of primary myelofibrosis, early, prefibrotic stage and overt myelofibrosis. The clinical manifestations of the prefibrotic stage are diverse, with the isolated finding of thrombocytosis reminiscent of essential thrombocythemia and prepolycytemic stages of polycythemia vera. The role of IFN-α in the early stages of primary myelofibrosis has recently been further intensified, and recent clinical studies have once again highlighted the potential of interferon to induce regression of bone marrow fibrosis. The article aims to draw the readers‘ attention to the importance of correctly distinguishing the prefibrotic stage of primary myelofibrosis from apparent primary myelofibrosis and other classic subtypes of myeloproliferative neoplasia.

Keywords: primary myelofibrosis, prefibrotic stage, overt myelofibrosis