Retrospective analysis of clinical data from 171 low-income patients with hemophilia in Shandong Province

To enhance attention on low-income patients with hemophilia, it is essential to urge governments and coagulation factor manufacturers to increase their investment in hemophilia care. This would ensure that low-income patients receive an adequate supply of clotting factors for both replacement therapy and prophylaxis. Clinical data from 171 low-income hemophilia patients were collected in Shandong Province between January 2017 and December 2019. "Low-income" is defined as having a per capita disposable income below the average for the years 2017 to 2019 in Shandong Province. The data collected included age, education, activity levels of factor VIII/IX and anti-factor VIII/IX inhibitors, therapeutic regimen, viral infections, and annual bleeding rate (ABR). Additionally, the translated and validated Chinese version of the Haemophilia Quality of Life questionnaire for adults (Haem-QoL-A), Hemophilia Joint Health Scores (HJHS), and annualized consumption of factor VIII or prothrombin complex concentrate (PCC) were applied. In our study, a total of 171 male patients-131 adults and 40 children-were included, and the median age was 30 years. The average annual income of these patients was $446.9. Of these, 133 patients (77.8%) were diagnosed with hemophiliac arthropathy. Eight out of 150 patients had anti-factor VIII inhibitors, while only one patient had anti-factor IX inhibitors. Among the 171 patients, only six children received low-dose prophylaxis (10-15 IU/kg, 1-2 times per week), while the remaining 165 patients received only inadequate on-demand treatment. In terms of viral infections, 20 patients (11.7%) were infected with transfusion-associated viruses. The median annual bleeding rate (ABR) was 20 bleeding events per year (range: 2-100). The mean Haem-QoL-A score was 62.3 ± 14.5. The mean total Hemophilia Joint Health Score (HJHS) for children was 8.1 ± 7.1, and for adults with hemophilia A or B (HA/HB), it was 40.1 ± 20.0 (P < 0.05). The annual per capita factor dosage for the 171 low-income hemophilia patients was calculated to be 6,182 IU/year. Providing additional support and care for low-income patients is crucial. Early prophylaxis in children is particularly important for protecting joint health.