Effect of hypomethylating agent on myelodysplastic syndrome with myelofibrosis
Objective:To explore the clinical efficacy of hypomethylating agent(HMA)in the treatment of pa-tients with or without myelofibrosis(MF)in myelodysplastic syndrome(MDS).Methods:147 MDS patients ad-mitted to Shanxi Bethune Hospital from January 2017 to September 2023 were collected,including 50 patients with MF grade 0 and 97 patients with MF grade 1-3.Patients treated with HMA were divided into two groups ac-cording to the severity of myelofibrosis,27 cases in the non-fibrotic group(MF 0)and 59 cases in the fibrotic phase group(MF 1-3);patients with MF grade 1-3 were divided into two groups according to whether they re-ceived HMA,30 cases in the supportive treatment group and 59 cases in the HMA group.Age,sex,hematologi-cal parameters(white blood cell count,neutrophil count,hemoglobin,platelet count),proportion of bone marrow blasts,risk stratification according to IPSS-R and IPSS-M,WHO 2016 classification,gene mutations(TP53,ASXL1,U2AF1,DNMT3A,SF3B1,TET2,JAK2,RUNX1)and treatment efficacy were compared between the two groups.Results:There were no significant differences in age,white blood cell count,neutrophil count,hemoglobin,percentage of bone marrow blasts,risk stratification according to IPSS-R versus IPSS-M,and gene mutations between non-fibrotic and fibrotic groups(P>0.05),sex,platelet count,median overall survival and median progression-free survival,statistically significant(P<0.05),Seventy-four of these patients were analyzed for efficacy and there was no significant difference between subgroups(P>0.05);Supportive treatment vs HMA:There were no significant differences in age,sex,leukocyte count,neutrophil count,hemoglobin,and median o-verall survival versus median progression-free survival(P>0.05).Platelet count,proportion of bone marrow blasts,risk stratification according to IPSS-R and IPSS-M,WHO 2016 typing,and gene mutations(ASXL1,TET2)were statistically significant(P<0.05).Conclusion:Myelofibrosis in MDS is associated with poor prog-nosis.Treatment with HMA alone did not improve outcomes in patients with MDS and myelofibrosis,and treat-ment with HMA alone did not prolong overall survival and progression-free survival in patients with MDS and my-elofibrosis.