Clinical Characteristics And Prognosis Of Myeloid Malignancies With Myelofibrosis

Objective To investigate the effects of myelofibrosis on the efficacy,prognosis and progression of acute myeloid leukemia(AML)and myelodysplastic syndrome(MDS)by analyze the clinical features and prognostic factors of patients diagnosed with AML and MDS.To evaluate the prognostic efficacy of the prognostic score system devised for myeloid fibrosis and myelodysplastic syndrome for AML with myelofibrosis and MDS with myelofibrosis in China.Methods 1.The clinical information and prognosis of 257 patients diagnosed with AML with or without myelofibrosis or MDS with or without myelofibrosis were analyzed retrospectively.According to degree of myelofibrosis,divided into myelofibrosis group and without myelofibrosis group.The clinical characteristics of the two groups were compared and analyzed.2.To investigate the effect of myelofibrosis on the transformation of MDS to AML,and the impact of myelofibrosis on the therapeutic efficacy of primary AML and AML patients transformed from MDS.3.The patients of AML with myelofibrosis and MDS with myelofibrosis were stratified according to PMF-IPSS,DIPSS,IPSS-Chinese,DIPSS-Chinese,MYSEC-PM,MDS-IPSS,IPSS-R and WPSS.And the possible prognostic factors were statistically analyzed.Results 1.Clinical features and laboratory examination: In MDS with myelofibrosis,21patients(22.8%)had splenomegaly and 8 patients(8.7%)had hepatomegaly,in MDS without myelofibrosis,7 patients(7.8%)had splenomegaly and 1 patients(1.1%)had hepatomegaly,the difference was statistically significant(P=0.011,P=0.018).The median neutrophil count of patients with MDS with myelofibrosis was 1.010*109 /L,compared with1.475*109/L in MDS without myelofibrosis,the difference was statistically significant(P=0.04).62 patients(67.4%)with MDS with myelofibrosis had active cell proliferation in bone marrow biopsy,compared with38 patients(42.2%)with MDS without myelofibrosis,the difference was statistically significant(P=0.003).Among MDS with myelofibrosis,28 patients(30.4%)had good karyotype,33 patients(35.9%)had moderate karyotype,and 31 patients(33.7%)had poor karyotype,among patients with MDS without myelofibrosis,63 patients(70.0%)with good karyotype,11 patients(12.2%)with moderate karyotype,and 16 patients(17.8%)with poor karyotype,the difference was statistically significant(P<0.001).There were no statistically significant differences in gender,age,hepatomegaly,splenomegaly,peripheral blood white blood cell count,peripheral blood hemoglobin,peripheral blood platelet count,lactate dehydrogenase,alkaline phosphatase,bone marrow hyperplasia degree,chromosome karyotype between AML patients with or without myelofibrosis(P>0.05).2.The rate of complete remission: The rate of MDS without myelofibrosis transform to AML was 5.6%,compared with 15.2% in MDS with myelofibrosis,the difference was statistically significant(P=0.033).After a first-line standard-dose chemotherapy,the rate of complete remission(CR)in AML with MF-0 or MF-1 patients after one course of chemotherapy was 46.3%,compared with 0% in AML with MF-2 patients,the difference was statistically significant(P=0.012).The rate of CR in AML with MF-0 or MF-1patients after two course of chemotherapy was 61.2%,compared with12.5% in AML with MF-2 patients,the difference was statistically significant(P =0.009).3.Survival time: The median survival time of patients with myeloid malignancies without myelofibrosis was 28(1-58)months,and the median survival time of patients with myeloid malignancies with myelofibrosis was 14(1-54)months,the difference was statistically significant(P = 0.002).The 2-year survival rate of patients with MDS without myelofibrosis was 54.6%,and the 2-year survival rate of patients with MDS with myelofibrosis was 29.5%,the difference was statistically significant(P=0.001).The 1-year survival rate of patients with AML transformed from MDS without myelofibrosis was60.0%,the 1-year survival rate of patients with AML transformed from MDS with myelofibrosis was 8.2%,the difference was statistically significant(P=0.018).4.Prognostic analysis: PMF-IPSS,DIPSS,IPSS-Chinese,DIPSS-Chinese,MYSEC-PM,MDS-IPSS,IPSS-R,WPSS could not accurately predict the prognosis of patients diagnosed with AML/MDS with myelofibrosis.Increasing the classification of myelofibrosis can improve the prognostic efficacy of MDS-IPSS and WPSS.Splenomegaly(P=0.015),transfusion-dependent(P=0.008),fibrosis grade≥2(P<0.001),CRP>5.0 mg/L(P=0.032)had poor prognostic significance.Conclusion 1.The number of hepatosplenomegaly,neutrophilreduction,active cell proliferation,and poor karyotype in patients with MDS with myelofibrosis was higher than in patients without fibrosis.2.The rate of transformation of MDS to AML can be increased and the overall survival time would be shorten influenced by myelofibrosis.3.Myelofibrosis affects the treatment of AML patients.The more severe the fibrosis,the lower the complete remission rate after chemotherapy.4.There is currently no prognostic system for AML/MDS with myelofibrosis.Splenomegaly,transfusion dependence,fibrosis grade,and CRP are associated with prognosis,providing new options for prognosis and treatment.