The First Part Is The Clinical Characteristics And Prognosis Analysis Of Patients With Primary Myelofibrosis In Different Age Groups. The Second Part Is A Report Of A Case Of Rucotinib In The Treatment Of Chronic Neutrophil Leukemia

Objective To analyze the clinical characteristics and prognosis of patients with primary myelofibrosis(PMF)in different age groups.Methods The medical records of 1321 patients with PMF from August 1984 to April 2018 were retrospectively analyzed.The clinical features and prognosis were summarized and compared according to age groups.Results Of the 1321 patients with PMF,727(55.0%)were males and 594(45.0%)females,with a median age of 54(14-89)years.The patients were divided into three groups according to their age:241(18.2%)patients under 40 years old,659(49.9%)patients between 41 and 60 years old and 421(31.9%)patients over 60 years old.The median overall survival(OS)of 1321 patients with PMF was 115(95%Cl 80-150)months.According to the age group,the median OS of the three groups(<40y,41-60y and>60y)were not reached,255(95%Cl 116-394)and 48(95%Cl 40-56)months,respectively.The 10-year OS were 63.8%,60.8%and 22.6%,respectively(P<0.001).There was no significance between the group under 40 years old and the group between 41 and 60 years old(P=0.071).IPSS,DIPSS,DIPSS-Plus and DIPSS-Chinese prognostic scoring systems were used in different age groups,which displayed obvious prognostic significance in different age groups.626 patients could be classified according to the mutation results:127 patients with high risk and 499 patients with low risk.The median OS were 45 months in high risk group and 168 months in low risk group,respectively(P<0.001).In addition to the above prognostic scoring system and mutation grouping,some clinical features have different prognostic value in different age groups.For PMF patients younger than 40 years old,acute leukemia transformation and RBC transfusion were independent adverse prognostic factors.For PMF patients between 41 and 60 years old,female,PLT?100><109/L,bone marrow blasts?5%,without splenomegaly,acute leukemia transformation and RBC transfusion were independent adverse prognostic factors.For PMF patients over 60 years old,bone marrow blasts?5%and without splenomegaly were independent adverse prognostic factors.Conclusion In our center,the majority of PMF patients were young and middle-aged.The OS of patients under 40 years old was similar to that of patients between 41 and 60 years old.The OS of patients over 60 years old was poor.Each prognostic scoring system had obvious prognostic significance in different age groups.The prognostic value of distinct clinical features was different in each age group.