Hemophilia A and B with special reference to prevention and treatment of arthropathy

Abstract: In patients with severe hemophilia it is today possible to prevent the development of arthropathy by giving effective continuous prophylaxis from an early age, thus preventing the VIII:C or IX:C concentration from falling below 1% of normal. All the signs show that if a high-dose prophylactic regimen - that is, 3,000-5,000 U/kg body weight annually - is started as early as 1-2 years of age, the patients´ factor VIII/IX concentrations can be maintained at a level of at least 1% and bleeds and joint defects can be virtually eliminated, allowing patients to live more or less normal lives, both physically and socially. Prophylaxis is also largely responsible for the changing picture in orthopedic surgery in hemophilia today, with a decreasing need of operations, an increasing average age of the patients, and a change in the kinds of operations performed. Although its outcome is inferior to that obtained in arthrosis, hip joint replacement in hemophilia is a viable option and should be considered as it is of value in certain cases. Surgery may also be undertaken in HIV-positive hemophiliacs under similar premises as for HIV-negatives, though indications for major surgery require meticulous consideration. In young hemophiliacs with inhibitors, a patient group otherwise difficult to treat, radioactive synoviorthesis is an appropriate treatment. The method is characterized by its ease of performance and limited requirements, and in most cases results in a manifest decrease in the joint bleeding frequency. Key words: , prophylaxis, factor VIII/IX concentrates, orthopedic surgery, total hip replacement, HIV, radioactive synoviorthesis