Elevated expression of the EZH2 gene in CALR -mutated patients with primary myelofibrosis

AbstractPrimary myelofibrosis (PMF) is one of the BCR/ABL-negative myeloproliferative neoplasms (MPNs), characterized by the diffuse fibrous hyperproliferation, bone marrow osteosclerosis, extramedullary hematopoiesis, and marked splenomegaly. The patients with PMF have an insidious onset, a long duration of clinical course, and the deteriorated quality of life. It has been reported that theCALR gene 9 exon mutations were detected in 25 –30% PMF patients, particularly as high as 80% in theJAK2/MPL-negative ones. As the second most common mutation inBCR/ABL-negative MPNs,CALR mutation has been included in the latest World Health Organization (WHO) classification criteria as one of the main diagnostic criteria for both essential thrombocythemia (ET) and PMF. Moreover, theCALR mutations indicated a favorable prognosis, which the mechanism is still under investigation. It was demonstrated that a characterized high expression ofEZH2 andSUZ12 inCALR-mutated patients. TakingEZH2 as the research entry point, we initially discussed the mechanism that theCALR-positive patients with PMF exhibited a better prognosis in the current study.
Source: Annals of Hematology - Category: Hematology Source Type: research