Abstract

Hemophilia A is an inherited bleeding disorder caused by factor VIII (FVIII) deficiency. In most minor-to-moderate bleeding events, FVIII concentrates therapy can result in significant lifesaving. However, chronic FVIII concentrates therapy can cause FVIII inhibitors synthesis and replacement therapy to be inefficacious. Immunosuppressive therapy can suppress FVIII inhibitors synthesis and make FVIII concentrates therapy more responsive. This study aims to determine the effects of low-dose methylprednisolone (MP) on acquired FVIII inhibitors. A randomized clinical trial was conducted on 11 hemophilia A male patients with a high level of FVIII inhibitors. This pretest–posttest study design consists of an intervention group (six patients) who received 1 mg/body weight/day MP and a control group (five patients) who received a 100 mg/day sugar pill placebo for 6 weeks. Statistical analysis used the independent t-test and paired t-test. The results showed significant FVIII inhibitors rising in pretest–posttest mean comparison of both the intervention group (p = 0.001) and control group (p = 0.001). Further results showed the intergroups’ pretest mean comparison (p = 0.976) and intergroups’ posttest mean comparison (p = 0.034). It can be concluded that low-dose MP significantly suppresses the FVIII inhibitors synthesis pace within 6 weeks instead of inhibiting the FVIII inhibitors synthesis.

Key words: adjuvant therapy, factor VIII, factor VIII inhibitors, hemophilia A, immunosuppressive therapy, methylprednisolone