Hemophilia primary prophylaxis

O Intravenous factor replacement with recombinant or plasma-derived products to treat or prevent bleeding is the primary treatment hemophilia. Primary prophylaxis is defined as the regular administration of factor concentrates with the intention of preventing joint bleeds.4 The rationale for primary prophylaxis is that individuals with factor levels of greater than 0.02 unit/mL (2 IU/dL) rarely suffer from spontaneous bleeds and arthropathy. Therefore, to maintain a trough level above this might convert severe hemophilia to moderate disease, with the abolition of joint bleeds and the associated arthropathy.5... 

Primary prophylaxis is expensive, but historical cohorts show progressively better outcomes (e.g., joint function and radiologic appearances) with more treatment, and prophylaxis thus is recommended as optimal therapy by the WFH, the World Health Organization (WHO), the U.K. Haemophilia Centre Doctors Organization, and the National Hemophilia... 

Hemophilia patients may receive prophylactic factor concentrate therapy to prevent or decrease bleeding episodes or they may receive on-demand factor concentrate therapy in response to a bleeding episode. In addition, prophylaxis may be primary on secondary. Controversy exists over whether the benefits of prophylaxis justify the cost, as well as the appropriate time to initiate prophylaxis. 

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