| Objective:Through retrospective analysis of clinical data of 200 hemophilia hospitalized patients in Department of Hematology,Jiangxi Provincial People’s Hospital(First age of bleeding,first age of diagnosis,first bleeding site,production of inhibitors,infection of blood-borne related diseases,etc.),The analysis of the status of diagnosis and treatment of hemophilia patients in the region will provide guidance and help for future clinical diagnosis and treatment of hemophilia.Methods:We selected 200 cases of hemophilia hospitalized in the Department of Hematology,Jiangxi Provincial People’s Hospital during 2015.01-2017.12.The clinical data of 200 cases were retrospectively analyzed.result:1.Among the 200 hemophilia patients,167 cases are HA(83.5%),33 cases are HB(16.5%),and HA: HB=5.06:1.2.According to 167 cases of HA and 33 cases of HB patients during2015.01-2017.12,they are hospitalized for FVIII:C or FIX:C for the first time.Among the 167 patients with HA,56 patients were are light(33.5%),56 patients are moderate(33.5%),and 55 patients are heavy(33.0%);Among 33 patients with HB,10 cases are light(30.3%),11 cases are moderate(33.3%),and 12 cases are heavy(36.4%).3.According to Chinese traditional ages,there are 5 age groups: ≤6 years old,7-17 years old,18-40 years old,41-65 years old,>65 years old,Among them,patients aged 18 to 40 had the highest number of visits(94%),followed by 65(32.5%)aged 7to 17 years.4.Among the 167 HA patients,the first hemorrhage is 0.5-58 years old,and the median age is 1 year.The first confirmed age is 0.5-61 years old and the median ageis 3 years old.The difference between the age of first bleeding and the age of diagnosis is statistically significant(t=5.575,P=0.000),and there is a delay in diagnosis.5.Among the 33 HB patients,the first hemorrhage is 0.5-47 years old,and the median age is 1 year.The age of first diagnosis is 0.5-47 years old,and the median age is 4 years old.The difference between the age of first hemorrhage and the age of diagnosis is statistically significant(t=2.252,P=0.031),and there is a diagnostic delay in diagnosis.6.In HA patients,APTT is statistically significant between light and moderate,light and heavy,medium and heavy(P<0.05).7.In HB patients,there is a statistically significant difference in APTT between mild and moderate,mild and severe(P < 0.05).There is no significant difference in APTT between medium and heavy(P>0.05).8.In 167 patients with HA and 33 patients with HB,the first cause of hemorrhage are the most spontaneous.They are: HA 132 cases(79.04%),HB 25 cases(75.75%).Followed by traumatic,iatrogenic.9.In 167 patients with HA and 33 patients with HB,the first bleeding sites is the skin.They are HA69(41.56%)and HB11(42.31%).Followed by joints,were: HA 67cases(40.36%),HB9 cases(34.61%).10.Of the 167 patients with HA,123(73.65%)have articular bleeding and 44(26.35%)have no bleeding.There are a total of 145 joint hemorrhages: 14 cases shoulder joint(9.65%),24 cases elbow joint(16.55%),2 cases wrist joint(1.38%),13 cases hip joint(8.96%),and 61 cases knee joint(42.07).%),Ankle joints is 27 cases(18.62%),Toe joints is 4 cases(2.77%).11.33 cases of HB patients: joint bleeding is 25 cases(75.76%),no bleeding is 8cases(24.24%).There are 31 cases joint hemorrhages,including 3 cases shoulders(9.67%),2 cases elbow joints(6.45%),7 cases ship joints(22.58%),14 cases knee joints(45.16%),and 5 cases ankle joints(16.14%).12.Among the 200 patients with hemophilia,84 patients have completed blood group tests,of which 71 were HA and 13 were HB.The number of O-type persons accounted for a large proportion: HA31 cases(43.67%)and HB 6 cases(46.15%).13.Among the 200 patients with hemophilia,162 are screened for inhibitors,161(96.41%)for HA,and 1(3.03%)for HB.Among them,18(11.18%)are positive for inhibitors and all are HA patients.High titer inhibitors are 15(83.33%),and low titer inhibitors are 3(16.67%).conclusion:1.The number of HA and HB visits is the highest in the age group of 7 to 17 and18 to 40 years old;2.There is a difference between the first age of bleeding and the age of diagnosis in patients with HA and HB,and there is a delay in diagnosis.3.There are significant differences in APTT between mild and moderate,mild and severe cases in HA and HB patients(P<0.05).4.The first hemorrhagic cause of hemophilia patients is spontaneous bleeding.The site of the first hemorrhage is skin and joint bleeding,and as the age increased,joint involvement became more and more obvious,and the knee joint is the most frequently involved.5.Patients with HA and HB have more O-type blood than non-O-type blood.6.The positive rate of FⅧ inhibitors is slightly lower than that of domestic and foreign studies. |
PDF Full Text Request