Patients with severe hemophilia number only around 400,000 worldwide,but their lifespan has increased because of improved treatment,and with that the prevalence of the disease is increasing. Despitethe relatively small number of patients with severe hemophilia,the disease has an important socioeconomic effect because ofits distinctive feature: a level of clotting factor (factorVIII or factor IX) so low that without replacement therapy,frequent hemorrhages occur, most often in the ankle, knee, andelbow joints. These joint hemorrhages culminate in a severearthropathy, with signs of inflammation (as in rheumatoid arthritis)as well as degenerative damage (as . . . [Full Text of this Article]
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From the Department of Hematology and Van Creveld Clinic and the Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, the Netherlands.
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