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Types of prophylaxis for patients with hemophilia A or B

Types of prophylaxis for patients with hemophilia A or B
Type of treatment Definition
Episodic (on demand) treatment Replacement factor given at the time of bleeding
Continuous (regular) prophylaxis Prophylaxis given to prevent bleeding for at least 45 of 52 weeks (85%) of a year
  • Primary prophylaxis
Continuous prophylaxis started before age three years and before the second large joint bleed
  • Secondary prophylaxis
Continuous prophylaxis started after two or more large joint bleeds but before the onset of chronic arthropathy
  • Tertiary prophylaxis
Continuous prophylaxis started after the onset of arthropathy to prevent further damage
Intermittent (periodic) prophylaxis Prophylaxis given to prevent bleeding for short periods of time such as during and after surgery
Replacement factor includes plasma-derived or recombinant factor concentrates and can be used for prophylaxis and/or treatment of bleeding episodes. Non-factor agents such as factor VIII mimetics, anti-TFPI therapies, emicizumab, marstacimab, and concizumab are only used for prophylaxis. Refer to UpToDate for indications and details of factor administration and additional therapies for patients with hemophilia.
Adapted from:
  1. Srivastava A, Brewer AK, Mauser-Bunschoten EP, et al. Guidelines for the management of hemophilia. Haemophilia 2013; 19:e1.
  2. Blanchette VS, Key NS, Ljung LR, et al. Definitions in hemophilia: Communication from the SSC of the ISTH. J Thromb Haemost 2014; 12:1935.
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