| Objective: Aim to investigate the basic situation,clinical characteristics,concurrent diseases,treatment and life status of patients with inherited coagulation disorders in Dalian City,and further to explore the ways to improve the status of survival,diagnosis and treatment of patients with inherited coagulation disorders.Methods: A retrospective analysis of the clinical data on the incidence of disease,bleeding condition,joint situation,related complications,prevention measures and life status of patients with hereditary coagulation disorders registered in Dalian Hemophilia Center from June 2010 to January 2018.Results: 151 patients with hereditary coagulation disorders were registered and diagnosed in Dalian Haemophilia centre.Including 111 patients with HA,30 patients with HB,3 patients with VWD,3 patients with F deficiency disease,1 patient with F XII deficiency,2 patients with of low FIB level,1 patient with hereditary coagulation factor deficiency.1.52 patiens(46.8 %)of HA have family history,meanwhile 10 patiens(33.3 %)of HB have family history.2.Among the 111 HA patients,46 patients(41.4%)were severe type,39 patients(35.1%)were moderate type,26 patients(23.4%)were mild type.Among the 30 HB patients,9 patients(30%)were severe type,,15 patients(50%)were moderate type,and 6 patients(20%)were mild type.3.The median age of the first hemorrhage of HA patients was 2.625 years old,and the first diagnosis of HA patients was 3.125 years old.The median age of the first hemorrhage of HB patients was 2.5 years old,and the first diagnosis of HB patients was 3 years old.There was no statistically significant difference in the median age of primary hemorrhage and first diagnosis between HA patients and HB patients.However,there were statistical significance between the age of the first hemorrhage and the first diagnosis of different types of HA and HB Respective.(severe type,moderate type,mild type)4.78 cases(70.3 %)of HA patients had joint hemorrhage,and 38 cases(34.2%)had haemorrhage of important organs.17 cases(56.7 %)of HB patients had joint hemorrhage,and 10 cases(33.3 %)had haemorrhage of important organs.There was no significant statistically difference in the hemorrhage rate of joint and important organs between HA and HB patients.Of the 10 patients with hereditary coagulation disorders,2 patients(20 %)had Massive hemorrhage of childbirth,and 1(10 %)had recurrent hematuria due to bladder cancer.There was no haemorrhage of important organs in the rest 7 patients with other hereditary coagulopathy.5.48 HA patients(43%)with with joint deformity,of which 17(15.3%)patients had Joint deformity of more than two parts,the median age of HA patients with joint deformity is 13 years old.11 HB patients(36.7%)with with joint deformity,of which 2(6.7%)patients had Joint deformity of more than two parts,the median age of HB patients with joint deformity is 15 years old.There is no statistical difference in the incidence of joint deformity between HA and HB patients.There were no joint deformities in all the other patients with hereditary coagulation disorders.6.7 cases of HA patients had never received treatment,20 cases(18%)of HA patients received treatment only in emergency bleeding,74 HA patients(66.7%)were treated on demand,and 10 HA patients(9%)were treated on prophylactic treatment.All the 30 hemophilia B patients have received treatment,10 HB patients(33.3%)have received treatment only in emergency bleeding,18 HB patients(60%)were treated on demand,and 2 HB patients(6.7%)were treated on prophylactic treatment.The rest of the patients with hereditary coagulation disorders were treated with fresh frozen plasma or cold precipitation for severe bleeding.7.14 cases of HA patients(12.6%)select cold precipitation as the main treatment method,60 hemophilia A patients(54.1%)select the infusion of human coagulation factor VIII as the main treatment method,30 hemophilia A patients(27%)select recombinant factor VIII as the main treatment method.3 HB patients(10%)were mainly treated with plasma infusion,meanwhile 27 HB patients(90%)were mainly treated with Human Prothrombin Complex concentrates.No HB patients select recombinant factor ? as the main treatment method.The rest of the patients with hereditary coagulation disorders were mainly treated with Blood products.8.The detection of infection assoiated with blood transfusion showed that HBV infection were 4 cases(3.6%),HCV infection were 5 cases(4.5%)syphilis infection was 1 cases(0.9%)in HA patients,and no HIV infection in HA patients,the total infection rate was 9.1% in HA patients.The detection of infection assoiated with blood transfusion showed that HBV infection were 2 cases(6.7%)in HB patient,and no HB patient with infection of syphilis and HIV,the total infection rate was 10%.There was no related infection in the other patients with hereditary coagulation disorders.45 cases of HA patients have received Coagulation factor VIII inhibitor detection,3 of them were positive.10 patients with HB were have received coagulation factor inhibitor detection,no patient was positive.The infection time of the above cases were all before 2010.9.Among the HA patients over 18 years old,41(29.2%)patients with junior middle school education and below,44 non-student patients(33.1%)aged 18-60 years had no work.Among the HB patients over 18 years old,5(29.4%)patients with junior middle school education and below,5 non-students patients(35.7%)aged 18-60 years had no work.Among the other hereditary coagulation abnormality patients over 18 years old,5 patients(50%)with junior middle school education and below,1 non-student patients(10%)aged 18-60 years had no work10.45(40.5%)HA patients spend more than 10 thousand yuan per year for disease,and the cost of hemophilia B which more than 10 thousand yuan per year for disease was 4 cases(13.3%).The cost of recurrent hematuria due to bladder cancer patient with VWD was more than 10 thousand per year.The average cost of the remaining hereditary coagulation disorders patients was not more than 1 thousand per year.8 patients died in the case collection period,and 5 patients died of bleeding,and the average life expectancy was lower than 60 years.11.8 patients died during the data collection period with an average life expectancy of less than 60 years old,and in which 5(62.5%)patients died of bleeding.Conclusion:1.There is a low diagnosis rate for the patients with hereditary coagulation disorders in Dalian city,and the difference between the age of first onset and the age of diagnosis suggests the existence of diagnostic delay.2.Most patients with hereditary coagulation disorders in Dalian city are mainly treated according to demand.Less prophylactic treatment leads to insufficient treatment and a high disability rate.3.The patients with hereditary coagulation disorders in Dalian city have the characteristics of low cultural level,high unemployment rate,high cost of disease treatment and poor quality of life.4.Prophylactic treatment significantly reduced the rate of joint deformity,suggesting that strengthening the prophylactic treatment of severe and moderate type patients can significantly reduce the disability rate.5.Dalian needs to increase the publicity of hereditary coagulation disease,thus improving cognition in medical workers and society,so as to reduce the incidence of hereditary coagulation disease. |
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