HEMOPHILIA PROPHYLAXIS IN YOUNG-PATIENTS - A LONG-TERM FOLLOW-UP

Objectives. To review long-term prophylactic factor treatment in young patients with severe haemophilia A and B, focusing on the orthopaedic and radiological outcome. Design. We received 34 patients with severe haemophilia A (n = 29) and B (n = 5), aged 7-22 years. Age at start o f treatment was 1-4.5 years. Dosages of factor concentrate (F VIII and F IX, respectively) were 25-40 IU/kg body weight, three times a week for haemophilia A and twice a week for haemophilia B. The patients had been checked annually over a 5-year period (1990-95). Orthopaedic and radiological joint scores were evaluated according to recommendations by the World Federation of Haemophilia. Setting. All results were obt ained at the Department for Coagulation Disorders, University of Lund, Malmo University Hospital, Malmo, Sweden. Results. Orthopaedic and ra diological joint scores were found to have remained unchanged during f ollow-up in almost all patients and to be still zero (i.e. no unaffect ed joints) in 79% (n = 27) of the patients. Conclusion. There is a gro wing international consensus haemophilic arthropathy can be prevented by administering early high-dose prophylaxis. The results of the prese nt investigation strongly support this opinion.