Résumé
Objective To observe the CALR mutation in patients with Ph negative chronic myeloproliferative neoplasms(MPNs) and its clinical significance. Methods From January 2012 to January 2015,the clinical data of ninety-seven patients with chronic myeloproliferative neoplasms was retrospectively analyzed and followed up to analyze different types of MPNs, including the clinical characteristics and gene mutation of polycythemia vera(PV),essential thrombocythemia(ET)and primary myelofibrosis (PMF).The hematological parameters and prognosis of patients with different mutation types were compared ( Cox regression model). Results Among the patients,the incidence of JAK2 mutation was the highest,64. 95% (63/97), followed by CALR mutation ( 19. 59% ( 19/97 ) ) and triple negative ( 10. 31% ( 10/97 ) ) . The incidence of MPL mutation was 5. 15% (5/97),which was the lowest and CALR mutations in ET and PMF were 28. 57%(10/35) and 28. 13% (9/32),respectively. The difference was not statistically significant (χ2 =1. 616,P>0. 05);the CALR gene mutation was not detected in PV patients. Compared with the JAK2 mutation, the hemoglobin,leukocyte and neutrophils in the patients with CALR mutation were lower (P<0. 05),PLT levels were lower in CALR-mutant ET patients ( P<0. 017) ,whereas platelet levels in CALR-mutant PMF patients were higher (P<0. 017). The incidence of disease progression in JAK2 and CALR mutation was 47. 62% (30/63)and 31. 58% (6/19) (χ2=1. 525,P>0. 05). The risk of disease progression in patients with CALR mutation was significantly lower than that of JAK2 mutation ( HR=0. 46,95%CI 0. 26-0. 98,P<0. 05) . Conclusion The clinical characteristics of MPNs patients with different gene mutations are different. The prognosis of MPNs patients with CALR mutation is better than that of JAK2 mutation.