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

Aim: To increase the attention given to low-income hemophilia patients, and to reduce the annual bleeding rate and incidence of hemophilia arthropathy, ultimately improving quality of life. Methods: Clinical data from 171 low-income hemophilia patients were collected from January 2017 to December 2019 in Shandong Province. The data includes age, education, employment status, activity of factor Ⅷ/Ⅸ and inhibitor, therapeutic regimen, virus infection, and annual bleeding rate (ABR). 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 FⅧ or prothrombin complex (PCC) consumption were applied. Results: 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' families was ¥6181.52. Among the patients with initial bleeding, the median age was 1 year. A total of 133 (77.8%) patients were diagnosed with hemophiliac arthropathy. Eight out of 150 patients had a hemophilia A inhibitor, while only 1 patient had a hemophilia B inhibitor. Among the 171 patients, only 6 children received low-dose prophylaxis. In terms of viral infections, twenty patients (11.7%) were infected with transfusion-associated viruses. The median ABR was 20 bleeding events/year (2-100). The mean Haemo-QoL-A score was 62.3\(\pm 14.5\). The mean total HJHS in children was 8.1\(\pm 7.1\), and that in adults with HA/HB was 40.1\(\pm 20.0\) (P<0.05). The annual per capita factor dosage of the 171 low-income hemophilia patients was calculated to be 6182 IU/year. Conclusions: In low-income hemophilia patients, the incidence of joint deformity is greater, the incidence of hemophilia inhibitors is lower, and the rate of virus infection through blood transfusion is lower; these findings are attributed to nonstandardized treatment. Moreover, the annual factor consumption of low-income patients in Shandong Province is significantly below the national average. Consequently, it is imperative to provide additional assistance and care to low-income patients. Early prophylaxis in children is essential to protect the joints.