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  • Title: The maintenance of tolerance after successful immune tolerance induction in hemophilia A and B: the North American Registry. Factor VIII/IX Subcommittee of the International Society for Thrombosis and Hemostasis.
    Author: DiMichele D, Kroner B, Factor VIII/IX Subcommittee of the International Society for Thrombosis and Hemostasis.
    Journal: Haematologica; 2000 Oct; 85(10 Suppl):40-2; discussion 42-4. PubMed ID: 11187869.
    Abstract:
    The North American Immune Tolerance Registry (NAITR) was initiated with the goal of determining, by questionnaire, immune tolerance (ITT) practices in hemophilia treatment centers in Canada and the United States. Sixty-eight centers (40%) responded. Of the 130 registry subjects with hemophilia A who completed ITT, 93 (72%) achieved tolerance. Of the 11 completed ITT courses in patients with hemophilia B, 4 (36%) were successful. Maintenance therapy was defined as any dotting factor regimen administered subsequent to the patient achieving the treating physician's criteria for successful immune tolerance. Seventy-five (81%) of 93 individuals in the hemophilia A cohort who successfully achieved tolerance were maintained on a regular (prophylactic factor VIII (FVIII) regimen for a variable time period post-ITT. The median dose used was 150 units/kg/week (range: 17-700). Forty-eight (64%) subjects remained tolerant while receiving regular doses of FVIII for a median observation period of 13 months (range 0-129 months). Of 27 patients whose maintenance therapy had been stopped, 17 (68%) remained tolerant over a median period of 19 months (range 1-54 months) and 9 relapsed. Among the relapses, 3 occurred after maintenance therapy was stopped; 6 were noted on prophylactic FVIII at a median time of 11 months (range 2-61 months). The definition of tolerance was reviewed for the 9 subjects who relapsed and was defined by a normal recovery and survival in only 1/9 patients. Among the 11 hemophilia B subjects in the cohort who completed tolerance, 4 had a successful outcome. Four individuals were placed on maintenance regimens of 25-100 units FIX/kg/day and all remained tolerant.
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