Clinical Characteristics Of 223 Patients With Hemophiliain Hemophilia Center Of Gansu Province

Objective:To analyze the clinical characteristics,diagonsis and treatment of hemophiliac in GanSu province.Methods:A retrospective study was conducted with 223 hemophiliac of our center between January 2010 and May 2015.Results:We identified 223 hemophiliac from 14 cities in GanSu and neighboring provinces, including 203 hemophilia A(HA) and 20 hemophilia B(HB), 222 patients were male, only one HA was women.Rural patients were 177 cases(79.4%),Urban patients 46 cases(20.6%).The median age of first bleeding 2 years of age, and confirmed the average age of 5.6 ±6.5 years, HA and HB delay time of diagnosis were 2.50±4.91 years and 2.07 ±4.76 years. All of the patients in the combined joint hemorrhage were 168 cases(75.3%), joint deformities wre 123 cases(55.2%),There were 82 cases of patients with more than two joint deformities, accounting for 36.8%.91.6% of the patients were on-demand treatment, HBV and HCV infection rates were 1.7% and 6.2%.86.9% of the patients first-visited hospital was below of tertiary hospitals, of which only 15.9% patients were considered hemophiliac by the initial clinician.Conclusion:The accurate diagnosis rate of hemophiliacs in GanSu province is low, the diagnosis delay time is longer, more merger joint hemorrhage, accumulative total joint deformity ratio is high, HCV infection rate is high. The physician at fist vitived has poor understanding of hemophilia.