MILD HEMOPHILIA IN CHILDREN - PREVALENCE, COMPLICATIONS, AND TREATMENT

Purpose: To review the natural history of mild hemophilia (factor Vm o r IX level >5% and <50%), including presentation and diagnosis, charac teristics of bleeding episodes, and therapy, at two hemophilia treatme nt centers. Methods: Inpatient and outpatient records of 55 patients < 17 years old with factor VIII or IX levels of 5 to 50% were reviewed a nd bleeding episodes for which medical attention was sought were analy zed. Results: Five of the 55 patients were gills. Girls and patients w ith no family history of hemophilia were diagnosed at 5.5 and 5.3 year s of age, respectively, compared to 2.8 years overall. Thirty-five pat ients were diagnosed because of a positive family history, No bleeding occurred in 18 patients; 190 bleeding episodes occurred in 37 patient s. Most bleeding occurred in muscle/soft tissue (101 episodes) or join ts (57 episodes) and were associated with trauma (174 episodes). Concl usions: Mild hemophilia may affect females more often than is apprecia ted. Delays in diagnosis and treatment may occur unless the variabilit y in presentation is recognized.