Criteria for the Management of Anti-Hemophilic Factors
1. The prescription must be written by a hematologist.
2. Anti-hemophiliac factors will be covered for indications approved by the FDA (refer to Table 1).
Table 1. Indications
Generic name
Brand name
Indications approved by FDA
Antihemophilic factor derived from human plasma.
Monoclate-P
Koate-DVI
Treatment of classic type A hemophilia, including perioperative management.
Hemofil M
Control and prevention of hemorrhagic episodes in classic hemophilia A.
Antihemophilic factor
Recombinant
Helixate FS
Kogenate FS
Control and prevention of bleeding in adults and children with hemophilia A
Perioperative management of hemophilia A
Prophylaxis to reduce bleeding episodes and reduce joint damage in patients with hemophilia A and no previous damage.
Recombinate
⪠Control and prevention of bleeding in adults and children with hemophilia A
⪠Perioperative management of hemophilia A
⪠Can be used to control bleeding in the presence of low levels of factor VIII inhibitors (<10 BU / mL) in hemophilia A Advate ⪠Control and prevention of bleeding in adults and children with hemophilia A ⪠Perioperative management of hemophilia A ⪠Routine prophylaxis to prevent / reduce episodes of bleeding in hemophilia A Refacto ⪠Control and prevention of hemorrhagic episodes and for surgical prophylaxis in patients with hemophilia A (congenital factor VIII deficiency or classic hemophilia). Anti-hemophilic factor Recombinant (Removal of Domain B Xyntha ⪠Control and prevention of bleeding in adults and children with hemophilia A ⪠Surgical prophylaxis for hemophilia A Factor IX, recombinant BeneFIX Rebinyn ⪠Control and prevention of bleeding in patients with hemophilia B, including peri-operative management Factor IX, recombinant Rixubis ⪠Control and prevention of bleeding episodes in adults with hemophilia B. ⪠Perioperative management in adults with hemophilia B. ⪠Routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adults with hemophilia B. Factor IX, derived from human plasma Mononine AlphaNine SD ⪠Prevention and control of bleeding in patients with hemophilia B Factor IX complex derived from human plasma. Bebulin Bebulin VH Profilnine SD ⪠Prevention and control of bleeding episodes in patients with hemophilia B Anti-coagulant inhibitor complex, plasma derivatives FEIBA NF FEIBA VH ⪠Control of spontaneous bleeding or for perioperative management of patients with hemophilia A or B and inhibitors Factor VIIa, recombinant NovoSeven RT ⪠Treatment of hemorrhagic episodes and perioperative management in patients with hemophilia A or B and inhibitors or with acquired hemophilia ⪠Treatment of bleeding episodes and peri-operative management in patients with congenital factor VII Complex antihemophilic factor derived from human plasma / von Willebrand factor Humate-P Alphanate ⪠Treatment and prevention of bleeding in patients with hemophilia A ⪠Treatment of bleeding in adults and children with von Willebrand disease, including spontaneous episodes or trauma-induced episodes ⪠Prevention of excessive bleeding during and after surgery where desmopressin is not effective or contraindicated ⪠Alphanate is not indicated for severe von Willebrand disease. Factor VII complex derived from human plasma with von Willebrand factor Wilate ⪠Treatment of spontaneous hemorrhagic episodes or trauma induced in patients with severe or mild / moderate von Willebrand disease, where desmopressin is not effective or contraindicated. Geneic Dose Calculation Anti-hemophilic factor derived from human and recombinant plasma Monoclate-P Koate-DVI Hemofil M Helixate FS Kogenate FS Recombinate Advate Refacto Xyntha The dose of the factor VIII concentrate is mainly based on the severity and location of the bleeding and the necessary levels of the circulation factor. In the absence of inhibitors, each factor VIII unit will increase endogenous factor levels by 2 IU / dL. The initial dose of the factor can be determined by: Desired dose (UI) = body weight (kg) x levels of factor x 0.5 intravenously. The administration rate should not exceed 3 ml / minute for adults and 100 IU / minute in young children. Circulating levels of factor VIII should be measured 15 minutes after the infusion. Subsequent doses are based on the recovery and half-life of factor VIII for each individual patient. Factor IX, derived from human plasma The recovery of factor IX is lower with recombinant factor IX compared to plasma-derived products, which require a higher dose. The initial dose can be calculated as: Adults Desired dose (IU) = Body weight (kg) x Factor level intravenously. 0.7 The administration rate should not exceed 3 ml / minute for adults and 100 IU / minute in young children. Factor IX, recombinant Rixubis One international unit of RIXUBIS per kg of bodyweight increased the circulating activity of factor IX by 0.9 international units / dL. Initial Dose: Required international units = body weight (kg) x desired factor IX increase (% of normal or IU / dL) x reciprocal of observed recovery (IU / kg per IU / dL). Maintenance dose depends: ⪠Type of bleed or surgery, the intensity of the hemostatic challenge, and number of days until adequate wound healing is achieved. Routine prophylaxis: 40 to 60 international units per kg twice weekly. Plasma-derived Factor IX complex Bebulin VH Profilnine SD Bebulin: In the absence of inhibitors, each factor IX unit will increase endogenous factor levels of 0.8 IU / dl. The initial dose of concentrate of the factor necessary to achieve the desired level can be determined by: Desired dose (UI) = Body weight (kg) x Levels of factor x 1.2 intravenously. Profilnine SD: In the absence of inhibitors, each factor IX unit will increase endogenous factor levels by 1 IU / dL. The initial dose of factor concentrates needed to achieve the desired level can be determined by: Desired dose (UI) = Body weight (kg) x Factor level intravenously. The administration rate should not exceed 2 ml / minute. Circulating levels of factor IX should be measured 15 minutes after infusion. Subsequent doses are based on the recovery and half-life of factor IX for each individual patient. Anti-coagulant inhibitor complex, plasma derivatives FEIBA NF FEIBA VH 50 to 100 IU / kg administered intravenously every 6 to 12 hours, depending on the severity and location of the hemorrhage. The administration rate should not exceed 2 IU / kg / minute. Factor VIIa, recombinant NovoSeven RT Hemophilia A or B with inhibitors: 90 mcg / kg every 2 hours as an intravenous bolus until hemostasis is achieved. Doses of 35 to 120 mcg / kg have been used in clinical trials. Congenital Factor VII Deficiency: 15 to 30 mcg / kg every 4 to 6 hours. Doses as low as 10 mcg / kg have been effective. Acquired hemophilia: 70 to 90 mcg / kg every 2 to 3 hours until hemostasis is achieved Complex antihemophilic factor derived from human plasma / von Willebrand factor Humate-P Alphanate Hemophilia A: The dose of factor VIII concentrate is mainly based on the severity and location of the bleeding and the necessary levels of circulation factor. In the absence of inhibitors, each factor VIII unit will increase the endogenous factor levels of 2 IU / dL. The initial dose of concentrate of the factor necessary to achieve the desired level can be determined by: Desired dose (UI) = Body weight (kg) x levels of factor x 0.5 intravenously. The administration rate should not exceed 3 ml / minute for adults and 100 IU / minute in young children. Circulating levels of factor VIII should be measured 15 minutes after the infusion. Subsequent doses are based on the recovery and half-life of factor VIII for each individual patient. Von Willebrand disease: Each unit of endogenous factor VIII will increase the von Willebrand factor by 5 IU / dl Treatment of bleeding episodes: 40 to 80 IU / kg every 8 to 12 hours intravenously. Subsequent doses are adjusted based on the severity and location of the hemorrhage. Perioperative management: One loading dose (eg, 50 to 60 IU / kg) followed by maintenance dose of approximately half the intravenous loading dose. Both the loading dose and the maintenance dose should be adjusted based on the type of surgery. The administration rate should not exceed 4 ml / minute of Humate-P and 10 ml / minute for Alphanate Factor VII complex derived from human plasma with von Willebrand factor Wilate A loading dose of 20 to 60 IU / kg, depending on the severity of the bleeding, followed by a maintenance dose of 20 to 40 IU / kg every 12 to 24 hours intravenously. 3. Prophylactic Therapy. a. Therapy. prophylactic with anti-hemophiliac factors may be considered in the following situations: ⪠Patient with severe hemophilia A or B defined by a level of coagulation factor <1%. ⪠Patients with repeated episodes of bleeding, mainly in the joints. Secondary prophylaxis can be considered for 4-8 weeks. ⪠Previous prophylactic therapy and after surgery or invasive procedure for up to 14 days. 4. Anti-hemophilic factors will be available for the management of the following cases a. Suspected bleeding in joints or muscles b. Any injury to the head, neck, mouth or eyes or evidence of bleeding in some of these areas. c. Unusual headache, particularly if it is followed by trauma d. Severe pain or swelling somewhere in the body and. Any open wound that requires closure by surgical or adhesive means f. History of an accident or trauma that may result in internal bleeding g. Any procedure or invasive surgery h. Persistent or profuse bleeding i. Gastrointestinal bleeding j. Fractures, dislocations or sprains Refer to Table 3 for plasma factor levels and duration of replacement therapy recommended by the World Federation of Hemophilia. Note: The calculated dose should be adjusted to the unit of the available vial and to the patient's response Recommendations of plasma level of factor and duration of administration1 Type of Hemorrhage Hemophilia A Hemophilia B Desired level Duration (Days) Desired level Duration (Days) Articulate 40% - 60% 1-2, maybe more if the answer is inadequate 40% - 60% 1-2, maybe more if the answer is inadequate Muscular (except iliopsoas) 40% - 60% 2-3, maybe more if the answer is inadequate 40% - 60% 2-3, maybe more if the answer is inadequate Iliopsoas • Initial • Treatment 80% - 100% 30% - 60% 1-2 3-5, sometimes more as prophylaxis during physiotherapy 60% - 80% 30% - 60% 1-2 3-5, sometimes more as prophylaxis during physiotherapy SNC / Head • Initial • Treatment 80% - 100% 50% 1-7 8-21 60% - 80% 30% 1-7 8-21 Neck and throat • Initial • Treatment 80% - 100% 50% 1-7 8-14 60% - 80% 30% 1-7 8-14 Renal 50% 3-5 40% 3-5 Deep laceration 50% 5-7 40% 5-7 Major Surgery • Preoperative • Postoperative 80% - 100% 60% - 80% 40% - 60% 30%-50% 1-3 4-6 7-14 60% - 80% 40% - 60% 30% - 50% 20%-40% 1-3 7-14 Minor Surgery • Preoperative • Postoperative 50%-80% 1-5, depending on the type of procedure 50%-80% 30%-80% 1-5, depending on the type of procedure
