Advances in understanding and management of polycythemia vera.

The discovery of a specific germline haplotype in JAK2 provided an explanation for the well known phenomenon of familial clustering of MPN. Clinical trials with JAK2 inhibitors, either specific or not, have been initiated and first results are available. However, the expectation that these drugs could selectively target mutant cells and cause a molecular remission, similar to the experience with imatinib in chronic myelogenous leukemia, has been largely unmet. With the aim to develop common tools for clinicians involved in conventional and experimental therapies, a set of criteria for evaluating response to treatment have been developed. SUMMARY:The last couple of years have witnessed significant improvements in understanding polycythemia vera biology and management, and the activation or completion of novel trials are expected to provide further information to improve the treatment.