For the documentation and comparison of outcomes of clinical care in hemophilia, especially with different treatment protocols, as well as in clinical trials of new clotting factor concentrates, it is necessary to have clear definitions of disease-related variables as well as the response to therapeutic interventions that are offered to treat or prevent bleeding. In 2001, White et al. [1] provided definitions for the severity of hemophilia and the levels for low and high response inhibitors on behalf of the Factor VIII and IX Subcommittee of the Scientific and Standardization Committee (SSC) of the International Society on Thrombosis and Haemostasis (ISTH). With increasing emphasis on assessment of outcomes and creating evidence for clinical practice in hemophilia, it became necessary to revisit definitions in hemophilia. To this end, the Factor VIII, Factor IX and Rare Coagulation Disorders Subcommittee of the SSC of the ISTH established a Project Group to provide consensus definitions in the following areas: classification; inhibitors; regular factor replacement therapy (prophylaxis); bleeding (and re-bleeding) into joints and muscles; target joints; and response to therapy including surgical hemostasis.
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