Hemophilia is an X-linked genetic disorder caused by a deficiency or absence in one of the clotting factors in the plasma. Hemophilia A is caused by a deficiency or absence of factor VIII. Hemophilia B is caused by a deficiency or absence of factor IX.

 Von Willebrand disease is a genetic disorder caused by missing or defective von Willebrand factor (vWF), a clotting protein.

Factor products are also used to treat other hematologic conditions such as fibrin stabilizing factor deficiency, factor X deficiency, congenital factor VII deficiency, and Glanzmann’s thrombasthenia.

This policy refers to the following clotting factors and coagulant blood products:

Factor VIIa, recombinant (NovoSeven RT and Sevenfact)

Factor VIIa, recombinant (NovoSeven RT) may be considered medically necessary in adult or pediatric individuals for ANY of the following conditions:

• Congenital Factor VII Deficiency:
  • Diagnosis of congenital factor VII deficiency; and
  • Treatment of bleeding episodes or perioperative management; or
• Glanzmann Thrombasthenia:
  • Diagnosis of Glanzmann’s thrombasthenia; and
  • As an alternative to platelet transfusion; and
  • Treatment of bleeding episodes or perioperative management; or
• Hemophilia A:
  • Diagnosis of hemophilia A; and
  • Documentation of inhibitors (e.g., Bethesda inhibitor assay); and
  • Treatment of bleeding episodes or perioperative management; or
• Acquired Hemophilia A:
  • Diagnosis of acquired hemophilia A; and
  • Treatment of bleeding episodes or perioperative management; or
• Hemophilia B:
  • Diagnosis of hemophilia B; and
  • Documentation of inhibitors (e.g. Bethesda inhibitor assay); and
  • Treatment of bleeding episodes or perioperative management.

Factor VIIa, recombinant (Sevenfact) may be considered medically necessary in individuals 12 years of age and older for ANY of the following conditions:

• Hemophilia A:
  • Treatment and control of bleeding episodes in individuals with inhibitors; or
• Hemophilia B:
  • Treatment and control of bleeding episodes in individuals with inhibitors.

Antihemophilic factor VIII, human (Hemofil M and Koate-DVI)

Antihemophilic factor VIII, human (Hemofil M and Koate-DVI) may be considered medically necessary in adult or pediatric individuals for ANY of the following conditions:

• Hemophilia A:
  • Diagnosis of hemophilia A; and
  • For control and prevention of bleeding episodes or to perform emergency and elective surgery in individuals with hemophilia A.

Antihemophilic factor VIII/von Willebrand factor complex, human (Humate-P)

Antihemophilic factor VIII/von Willebrand factor complex, human (Humate-P) may be considered medically necessary for ANY of the following conditions:

• Hemophilia A in individuals 18 years and older:
  • Diagnosis of hemophilia A; and
  • Treatment and prevention of bleeding; or
• von Willebrand disease in pediatric and adult individuals
  • Diagnosis of von Willebrand disease:
    • Severe disease; or
    • History of failure, contraindication, or intolerance to treatment with desmopressin in those with mild to moderate disease; and
• One of the following:
  • Treatment of spontaneous and trauma-induced bleeding episodes; or
  • Prevention of excessive bleeding during surgery (i.e., surgical prophylaxis).

von Willebrand factor complex, recombinant (Vonvendi)

von Willebrand factor complex, recombinant (Vonvendi) may be considered medically necessary in individuals 18 years and older when the following criteria are met:

• von Willebrand disease:
  • Diagnosis of von Willebrand disease; and
  • One of the following:
    • On-demand treatment and control of bleeding episode; or
    • Perioperative management of bleeding.

Antihemophilic factor VIII/von Willebrand factor complex, human (Wilate)

Antihemophilic factor VIII/von Willebrand factor complex, human (Wilate) may be considered medically necessary in adult or pediatric individuals when the following criteria are met:

• Hemophilia A:
  • Routine prophylaxis to reduce the frequency of bleeding episodes; or
  • On-demand treatment and control of bleeding episodes; or
• von Willebrand disease:
  • Diagnosis of von Willebrand disease; and
  • One of the following:
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding.

Antihemophilic factor VIII, recombinant (Advate, Kogenate FS, Novoeight, Nuwiq, Recombinate, Xyntha, and Xyntha Solofuse)

Antihemophilic factor VIII, recombinant (Advate, Kogenate FS, Novoeight, Nuwiq, Recombinate, Xyntha, and Xyntha Solofuse) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia A:
  • Diagnosis of hemophilia A: and
  • One of the following:
    • Control and preventing of bleeding episodes; or
    • Perioperative management; or
    • Routine prophylaxis to prevent or reduce the frequency of bleeding episodes.

Antihemophilic factor VIII, recombinant, pegylated (Adynovate, Kovaltry, and Afstyla)

Antihemophilic factor VIII, recombinant, pegylated (Adynovate, Kovaltry, and Afstyla) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia A:
  • Diagnosis of hemophilia A: and
  • One of the following:
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding; or
    • Routine prophylaxis to reduce the frequency of bleeding episodes.

Antihemophilic factor VIII, recombinant, pegylated (Jivi)

Antihemophilic factor VIII, recombinant, pegylated (Jivi) may be considered medically necessary in previously treated individuals 12 years and older when the following criteria are met:

• Hemophilia A:
  • Diagnosis of hemophilia A; and
  • One of the following:
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding; or
    • Routine prophylaxis to reduce the frequency of bleeding episodes.

Antihemophilic factor VIII, recombinant, glycopegylayed-exei (Esperoct)

Antihemophilic factor, recombinant, glycopegylated-exei (Esperoct) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia A:
  • Diagnosis of hemophilia A; and
  • One of the following:
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding; or
    • Routine prophylaxis to reduce the frequency of bleeding episodes. 

Antihemophilic factor VIII, recombinant, Fc fusion protein (Eloctate and Altuviiio)

Antihemophilic factor VIII, recombinant, Fc fusion protein (Eloctate) and antihemophilic factor VIII, recombinant, Fc-VWF-XTEN fusion protein-ehtl (Altuviiio) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia A:
  • Diagnosis of hemophilia A; and
  • One of the following:
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding; or
    • Routine prophylaxis to reduce the frequency of bleeding episodes. 

Antihemophilic factor VIII, recombinant, porcine sequence (Obizur)

Antihemophilic factor VIII, recombinant, porcine sequence (Obizur) may be considered medically necessary in individuals 18 years and older when ALL of the following criteria are met:

• Acquired hemophilia A:
  • Diagnosis of acquired hemophilia A; and
  • For the on-demand treatment and control of bleeding episodes 

Coagulation factor IX (Mononine)

Coagulation factor IX (Mononine) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia B:
  • Diagnosis of hemophilia B; and
  • For the prevention and control of bleeding.

Coagulation factor IX (AlphaNine SD and Profilnine)

Coagulation factor IX (AlphaNine SD and Profilnine) may be considered medically necessary in individuals 17 years of age and older when the following criteria are met:

• Hemophilia B:
  • Diagnosis of hemophilia B; and
  • For the prevention and control of bleeding.

Coagulation factor IX, recombinant (BeneFIX, Rixubis, Idelvion) and coagulation factor IX, recombinant, Fc fusion protein (Alprolix)

Coagulation factor IX, recombinant (BeneFIX, Rixubis, Idelvion) and coagulation factor IX, recombinant, Fc fusion protein (Alprolix) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia B:
  • Diagnosis of hemophilia B; and
  • One of the following:
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding; or
    • Routine prophylaxis to reduce the frequency of bleeding episodes.

Coagulation factor IX, recombinant (Ixinity)

Coagulation factor IX, recombinant (Ixinity) may be considered medically necessary in individuals 12 years of age and older when the following criteria are met:

• Hemophilia B:
  • Diagnosis of hemophilia B; and
  • One of the following:
    • Control and prevention of bleeding episodes; or
    • Perioperative management.

Coagulation factor IX, recombinant, pegylated (Rebinyn)

Coagulation factor IX, recombinant, pegylated (Rebinyn) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia B:
  • Diagnosis of hemophilia B; and
  • One of the following:
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding.

Coagulation factor X, human (Coagadex)

Coagulation factor X, human (Coagadex) may be considered medically necessary in individuals when the following criteria are met:

• Hereditary factor X deficiency:
  • Diagnosis of hereditary factor X deficiency; and
  • One of the following:
    • Routine prophylaxis to reduce the frequency of bleeding episodes; or
    • On-demand treatment and control of bleeding episodes; or
    • Perioperative management of bleeding in individuals with mild and moderate hereditary Factor X deficiency.

Coagulation factor XIII concentrate, human (Corifact)

Coagulation factor XIII concentrate, human (Corifact) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Factor XIII deficiency:
  • Diagnosis of congenital factor XIII deficiency; and
  • One of the following:
    • Routine prophylactic treatment; or
    • Perioperative management of surgical bleed.

Coagulation factor XIII A-subunit, recombinant (Tretten)

Coagulation factor XIII A-subunit, recombinant (Tretten) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Congenital factor XIII A-subunit deficiency:
  • Diagnosis of congenital factor XIII A-subunit deficiency; and
  • For routine prophylaxis of bleeding.

Emicizumab (Hemlibra)

Emicizumab (Hemlibra) may be considered medically necessary in adult and pediatric individuals with or without inhibitors when the following criteria are met:

• Hemophilia A:
  • Diagnosis of hemophilia A: and
  • One of the following:
    • Routine prophylactic treatment of moderate hemophilia with documented need for prophylaxis treatment or severe status less than 1% of normal factor; or
    • Prophylactic therapy of documented history of one (1) or more episodes of spontaneous bleeding into joints.

Anti-inhibitor coagulant complex (FEIBA NF, FEIBA VH)

Anti-inhibitor coagulant complex (FEIBA NF, FEIBA VH) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Hemophilia A
  • Diagnosis of hemophilia A; and
  • Documentation of inhibitors (e.g. Bethesda inhibitor assay); and
  • One of the following:
    • Control and prevention of bleeding episodes; or
    • Perioperative management; or
    • Routine prophylaxis to prevent or reduce the frequency of bleeding episodes:
    • In individuals with moderate hemophilia with documented need for prophylaxis treatment or severe status less than 1% of normal factor; or
    • In individuals with a documented history of one (1) or more episodes of spontaneous bleeding into joints; or
• Hemophilia B
  • Diagnosis of hemophilia B; and
  • Documentation of inhibitors (e.g. Bethesda inhibitor assay); and
  • One of the following:
    • Control and prevention of bleeding episodes; or
    • Perioperative management; or
    • Routine prophylaxis to prevent or reduce the frequency of bleeding episodes:
      • In individuals with moderate hemophilia with documented need for prophylaxis treatment or severe status less than 1% of normal factor; or
      • In individuals with a documented history of one (1) or more episodes of spontaneous bleeding into joints; or

Fibrinogen concentrate (RiaSTAP, Fibryga)

Fibrinogen concentrate (RiaSTAP, Fibryga) may be considered medically necessary in adult and pediatric individuals when the following criteria are met:

• Factor I deficiency:
  • Diagnosis of congenital fibrinogen deficiency, including afibrinogenemia and hypofibrinogenemia; and
  • Treatment of acute bleeding episode.

Tranexamic Acid (Cyklokapron)

Tranexamic acid (Cyklokapron) may be considered medically necessary when the following criteria are met:

• Hemophilia A
  • Diagnosis of hemophilia A; and
  • Prevention of hemorrhage and reduction of the need for replacement therapy during and following tooth extraction or other surgeries (e.g., polypectomy etc.); and
  • Short-term treatment not exceeding eight (8) days; or
• Hemophilia B
  • Diagnosis of hemophilia B; and
  • Prevention of hemorrhage and reduction of the need for replacement therapy during and following tooth extraction or other surgeries (e.g., polypectomy etc.); and
  • Short-term treatment not exceeding eight (8) days.

Immune Tolerance Induction

High-dose immune tolerance induction may be considered medically necessary when ALL of the following criteria are met:

• Member has a diagnosis of hemophilia A or hemophilia B; and
• Member has inhibitors (anti-FVIII and anti-FIX allo-antibodies);and
• Dosing continues beyond 14 days.

Immune tolerance induction limitations:

• Continued immune tolerance induction is considered not medically necessary when ALL of the following criteria are met:
  • Anti-hemophilic factor or factor IX survival after infusion is normal (6-hr level at least 50% of 10-min level); and
  • Inhibitor levels become undetectable; and
  • Recovery of anti-hemophilic factor or factor IX levels after infusion is normal (defined as at least 85 % of the expected for individuals without inhibitors).  
• Cases in which members are on immune tolerance induction for six (6) months or more must have documentation of referral to and evaluation by a hemophilia treatment center to determine whether continued immune tolerance therapy is medically necessary; and 

Alprolix, Idelvion, Ixinity, Rixibus and Hemlibra are not indicated for induction of immune tolerance.

The use of hemophilia treatment clotting factors/coagulant blood products not meeting the criteria as indicated in this policy is considered not medically necessary.