Prophylactic transfusion for hypertrophic synovitis in children with hemophilia.

Abstract

A 7- to 9-month protocol of prophylactic transfusion was used to treat 33 joints in 19 children with severe hemophilia (< 1 U/dL Factors VIII or IX) and hypertrophic synovitis. The overall rate of hemarthrosis was reduced, but only 36% (12 of 33 joints) achieved a good result (defined as 0-0.5 bleeding episodes per month and decreased synovial hypertrophy 1 year after completing treatment). Age and severity of arthropathy at initiation of treatment did not affect the result. The degree of synovial hypertrophy and involvement of the knee joint showed an adverse trend, but these factors did not achieve statistical significance. The number of episodes of breakthrough bleeding during the first 6 weeks of therapy was significantly associated with a poor result. Based on the results of this study, a trial of transfusion therapy is recommended for recurrent hemarthroses and synovitis in patients with hemophilia, but the duration of thrice weekly treatment has been increased and the duration of prophylaxis has been reduced in selected cases.