Inhibitor Neutralization and Continuous Infusion of Clotting Factor Concentrates for Hemophilia Patients with High-responding Inhibitor

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  • 血友病ハイレスポンダーインヒビター保有患者に対する凝固因子製剤によるインヒビターの中和と持続輸注療法

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We report successfully controlled severe bleeding in two hemophiliacs with a high responding titer. This was accomplished by neutralizing the inhibitors with a bolus infusion of a sufficient amount of the relevant blood coagulation factor concentrate, which was followed by a continuous infusion of the concentrate to maintain the titers of the factors in plasma at required levels.<br>The first patient, a 38-year-old man with severe hemophilia A, suffered from an intracerebellar hemorrhage. The inhibitor titer on his admission was 2.1 Bethesda units (BU)/ml. An initial bolus dose of 5,000 U of recombinant factor VIII (FVIII) concentrate was administered, followed by a continuous infusion of the concentrate at 3.8 U/kg/hr. Plasma FVIII : C level was maintained at 0.9-1.44U/ml over a period of 4 days, resulting in the reduction of the size of hematoma. At the age of 40, he suffered from a parietal lobe intracerebral hemorrhage. Despite the use of prothrombin complex concentrate (PCC) as a bypassing agent, the brain CT revealed a new hemorrhage in the subdural space. The inhibitor titer at this episode was 10 BU/ml. An initial bolus dose of 12, 000U of recombinant FVIII concentrate, followed by a continuous infusion of the concentrate at 4-6U/kg/hr raised the plasma FVIII : C level to 0.54-2.04U/ml over 5 days and resulted in the reduction the size of hematoma.<br>The second patient was a 4-year-old boy with severe hemophilia B. He was hurt on the right foot, having developed a large subcutaneous hematoma. The bypassing therapy with the prothrombin complex concentrate over a period of 6 days did not reduce the hematoma size. The inhibitor titer at the time of his admission was 2.1BU/ml. He was given an initial bolus dose of 2,000U of factor IX (FIX) concentrate, followed by a continuous infusion of the concentrate at 13U/kg/hr that yielded the FIX : C level at 1.3U/ml, and reduction in the size of the hematoma.

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