Radiologic evaluation of prophylaxis in severe haemophilia

Abstract

The effect of prophylactic treatment of severe haemophilia A and B was evaluated with a recently described radiologic score system for assessing the severity of arthropathy. The severity of the arthropathy was assessed in 50 patients with severe haemophilia receiving prophylaxis with factor VIII or IX (25--30 units factor VIII or IX/kg bodyweight at 4--7 day intervals), and in 9 in whom the condition was complicated by inhibitors and who were thus not receiving prophylaxis. The score values found in a recent investigation of the natural course of haemophilia served as controls in the evaluation of the effect of the prophylaxis. In the group with severe haemophilia receiving prophylaxis changes appeared only exceptionally in joints not affected already before the prophylaxis, and the course of the arthropathy and its severity according to age did not vary to any noteworthy extent from that in the control group with moderate haemophilia. In the cases complicated by inhibitors the joints were most often worse than those in the control group with severe haemophilia. It is concluded that prophylaxis has a considerable effect on the course of the arthropathy and the prophylaxis should be instituted at an early stage of the disease, when the joints are still unaffected.