The Inhibitor Development Of 93 Cases Of Hemophilia A Patient In Yunnan Province And The Risk Factors

Objective:Now, there are no unified epidemiological investigation conclusion about the producing rate of inhibitor and its impact factors for patients with hemophilia A in China. This study mainly discuss the inhibitor producing rate and the environmental aspects of the main influence factors for hemophilia A patients treated with FVIII in Yunnan province. Aim at realise the production reactor of FVIII inhibitor of patients with hemophilia A in Yunnan province and get a preliminary assessment of effect of the environmental factors about the inhibitor production, which will work to provide a reference for the related diagnosis and treatment for patients with hemophilia A, to reduce or avoid the unnecessary bleeding risk, improve the prognosis and the quality of life in patients with hemophilia A.Methods:Formulated a inhibitor associated risk factors questionnaire witch match the characteristics of hemophilia A patient in Yunnan province. Take a questionnaire investigation, telephone tracking, and regular follow-up for the hemophilia A patient who were take a registration in case management center administration of hemophilia on to the first affiliated hospital of kunming medical university. Collect the laboratory values of FVIII inhibitor qualitative and quantitative detection of the hemophilia A patient group. Make a statistical analysis about the questionnaire of clinical information and testing results of inhibitor using the SPSS22.0 statistical software. To understand the incidence of FVIII inhibitor and its environmental factors of hemophilia A patients taking a replacement therapy in Yunnan provence.Results:1、The distribution of the age of this group of patients:the age of this group of patients are fluctuated in 3-44 years of age (median age is 8.0 years), most patients on 2-20 years old.2、The distribution of the initial diagnosis age of This group of patients:the initial diagnosis age of this group of patients are fluctuated in 0-23 years of age (median age is 1.0 years), the majority of initial diagnosis age of children with hemophilia is concentrated between 1 to 2 years old.3、The FVIII inhibitor detection of this group of patients:there are 9 cases in 93 patients with FVIII inhibitor, inhibitor detection rate of 9.7%.3 cases with high drop degree,6 cases with low drop degree, the highest drop degree is 19.7 BU/ml. The inhibitor drop degree of this 3 cases of high drop was not change during the follow-up period, The inhibitor drop degree of this 6 cases of low drop continuous positive and stay in the low degree of state during the periodic review.4、The statistical analysis of relevant factors results suggest that Hemophilia severity and inhibitor have significant correlation (P=0.045< 0.045). The statistical analysis results also suggest that the FVIII preparations for the first time exposure dose exist significant correlation with the formation of inhibitor(p=0.04<0.05). At the same time, the statistical analysis results suggest that there are statistically significant differences of the cause of hemorrhage constitute between the positive and negative inhibitor groups (p= 0.009< 0.05).The result suggest that inhibitor positive group of patients with high bleeding risk than inhibitor negative groups. There are no significant statistical correlation between different preventive treatment, exposure, and infection with inhibitor produced.Conclusion:Inhibitor of this group of patients was 9.7%, of which the high and low degree of patients with a ratio of 1:2, consistent with the domestic and foreign related research reports. The statistical analysis of relevant factors results suggest that Hemophilia severity and inhibitor have significant correlation. The incidence of inhibitors of heavy hemophiliac is higher than of the light and medium hemophiliacs. The statistical analysis results also suggest that the FVIII preparations for the first time exposure dose exist significant correlation with the formation of inhibitor. At the same time, the statistical analysis results suggest that there are statistically significant differences of the cause of hemorrhage constitute between the positive and negative inhibitor groups. The result suggest that inhibitor positive group of patients with high bleeding risk patients than inhibitor negative groups. The prevention treatment whether or not can reduce the incidence of inhibitor remains to be further large sample research. Through this statistical analysis we know that the normative and strength of diagnosis and treatment of the patients in our hemophilia patients center far did not reach its strength and standardization, the future work on diagnosis and treatment of hemophilia patients will need to be strengthened.