Research progress of myeloproliferative neoplasms / 中国实验血液学杂志

ABSTRACT

The classic BCR-ABL negative myeloproliferative neoplasm (MPN) comprise polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). These three disorders are characterized by stem cell-derived clonal myeloproliferation and presence of somatic mutations. The WHO diagnostic criteria for the classic BCR-ABL negative MPN has been revised in the 2008 edition by incorporating new information about their molecular pathogenesis. Robust prognostic system for PMF has already done, and those for PV and ET are under discussion. Treatment with novel drugs is promising, and allo-stem cell transplantation (allo-ASCT) is the only curative treatment for myelofibrosis, however, the patient selection and management before transplant have been discussed for a long time.