When should prophylactic treatment in patients with haemophilia A and B start?--The German experience

摘要: Radiological and orthopaedic outcome in severe moderate haemophilia A B patients undergoing long-term prophylactic treatment were prospectively investigated focusing on the age of onset prophylaxis number joint bleedings prior to treatment. We report 21 with receiving between 3.1 16.1 years' duration. Three patient groups evaluated according at prophylaxis. In group I was initiated first 2 years life. Patients II received 3-6 years. Late-onset or secondary started 6 above seven (group III). All virus-inactivated F VIII IX concentrates dosages 30-50 IU/kg body weight i.v. three times per week for those twice B. Elbow, knee ankle joints 3-4-yearly intervals radiological scores recommended by World Federation Haemophilia. The total before after start recorded all patients. 7/8 had unaffected constant zero 1, a median 11.25 One this demonstrated mild alterations (score 4). showed neither nor study entry. Surprisingly, worsening could be detected despite ongoing 3-year interval (median score 4, 8). Treatment III already considerable damage entry 11 (0-33) 4 (0-11). Despite treatment, both 19.5, range 2-47) 8, 2-12) deteriorated 3 Prior prophylaxis, no only one bleeding occurred I. II, six bleeds (range 1-8) reported started. usually experienced more than 10 haemorrhages 6-10 more). Under decreased significantly III. However, increased as sign progressing osteoarthropathic reporting five whereas assessed episode Even small seems cause irreversible leading haemophilic arthropathy. Once apparent, further progression not arrested (groups order prevent arthropathy, effective should least