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Coagulation Factors
08.00.92ak

Policy

The Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition.

​​MEDICALLY NECESSARY

HEMOPHILIA A (FACTOR VIII DEFICIENCY)
The following coagulation factors are considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with hemophilia A (factor VIII deficiency):
  • Human preparations: Alphanate, Koate-DVI, Hemofil M, Humate-P, and Wilate
  • Recombinant preparations: Advate, Adynovate, Afstyla, Altuviiio, Eloctate, Esperoct, Jivi, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, and Xyntha/Xyntha Solofuse​
  • Human monoclonal antibody: Alhemo, Hemlibra​, Hympavzi
  • Small interfering ribonucleic acid: Qfitlia 
HEMOPHILIA B (FACTOR IX DEFICIENCY)
The following coagulation factors are considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with hemophilia B (factor IX deficiency):
  • Human preparations: Alphanine SD, Profilnine  ​
  • Recombinant preparations: Alprolix, BeneFI​X, Idelvion, Ixinity, Rebinyn, and Rixubis​
  • Human monoclonal antibody: Alhemo, Hympavzi​​
  • Small interfering ribonucleic acid: Qfitlia ​
HEMOPHILIA C (FACTOR XI DEFICIENCY)
Factor XI concentrates are not currently available in the United States.

HEMOPHILIA WITH INHIBITORS TO FACTORS VIII OR IX
The following coagulation factors are considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with hemophilia with inhibitors to factors VIII or IX:
  • Human and recombinant factors VIII or IX (see hemophilia A and B above)
  • FEIBA (human plasma–derived activated prothrombin complex concentrate [APCC])
  • Recombinant activated coagulation factor VIIa (e.g., NovoSevenRT, Sevenfact)
  • Human monoclonal antibody
    • Alhemo for hemophilia with inhibitors to factor VIII, or IX
    • Hemlibra for hemophilia with inhibitors to factor VIII
  • Small interfering ribonucleic acid: Qfitlia 
​FACTOR I DEFICIENCY, ACQUIRED  
Fibryga (fibrinogen concentrate [Human]) is considered medically necessary and, therefore, covered for the treatment of bleeding episodes in individuals with​ acquired fibrinogen deficiency (AFD).

FACTOR I DEFICIENCY, CONGENITAL
Fibryga and RiaSTAP (fibrinogen concentrate [Human]) are considered medically necessary and, therefore, covered for the treatment of bleeding episodes in individuals with congenital fibrinogen deficiency, including afibrinogenemia and hypofibrinogenemia. NOTE: See EXPERIMENTAL/INVESTIGATIONAL section regarding dysfibrinogenemia.

FACTOR VII DEFICIENCY, CONGENITAL
NovoSeven RT (recombinant-activated coagulation factor VIIa) is considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with congenital factor VII deficiency.

FACTOR VIII DEFICIENCY, ACQUIRED
The following coagulation factors are considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with acquired factor VIII deficiency:
  • Factor VIII concentrates, including Obizur
  • Alphanate (antihemophilic factor VIII/von Willebrand factor complex [human])
  • NovoSeven RT (recombinant-activated coagulation factor VIIa)
  • FEIBA (human plasma–​derived APCC)​
FACTOR X DEFICIENCY, ACQUIRED OR CONGENITAL
Coagadex is considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with factor X deficiency

FACTOR XIII DEFICIENCY, ACQUIRED OR CONGENITAL
The following coagulation factors are considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with acquired or congenital factor XIII deficiency:
  • Corifact​ (factor XIII concentrate [Human]) 
  • Tretten (factor XIII A-subunit [Recombinant]) ​
GLANZMANN THROMBASTHENIA (GLYCOPROTEIN COMPLEX IIb/IIIa DEFICIENCY)
NovoSeven RT (recombinant-activated coagulation factor VIIa) is considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with Glanzmann thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets.

VON WILLEBRAND DISEASE
The following coagulation factors are considered medically necessary and, therefore, covered for the prevention and/or treatment of bleeding episodes in individuals with von Willebrand disease when the use of desmopressin (1-desamino-8-D-arginine vasopressin [DDAVP]) is known or suspected to be ineffective or contraindicated:
  • Human preparations: Alphanate, Humate-P, Wilate
  • Recombinant preparation: Vonvendi​

EXPERIMENTAL/INVESTIGATIONAL

In addition to indications that are not described above in the Medically Necessary section, Fibryga and RiaSTAP are also considered experimental/investigational for the treatment of dysfibrinogenemia and, therefore, not covered because the safety and/or effectiveness of this service cannot be established by review of the available published peer-reviewed ​literature.

All other uses for coagulation factors other than US Food and Drug Administration–​labeled indications are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the Company medical policy on off-label coverage for prescription drugs and biologics.

NOT ELIGIBLE FOR REIMBURSEMENT

Bebulin, Helixate FS, Hyate:C, Monoclate-P, Mononine, NovoSeven, Profilnine SD​, and ReFacto are no longer manufactured and have been withdrawn from market; therefore, they are not eligible for reimbursement.


REQUIRED DOCUMENTATION​

The individual's medical record must reflect the medical necessity for the care provided. These medical records may include, but are not limited to: records from the professional provider's office, hospital, nursing home, home health agencies, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider. All documentation is to be available to the Company upon request. Failure to produce the requested information may result in a denial for the service.

BILLING REQUIREMENTS

If there is no specific HCPCS code available for the drug administered, then the drug must be reported with the most appropriate unlisted code along with the corresponding National Drug Code (NDC).

Guidelines

BENEFIT APPLICATION

Coverage is subject to the terms, conditions, and limitations of the member's contract.

FOR MEMBERS ENROLLED IN PENNSYLVANIA PRODUCTS ​

The majority of coagulation factor products can be self-administered by the member or the caregiver in their home. For most of the Company's Commercial Plans, self-administered drugs are not covered under the Medical Benefit and are only eligible for coverage under the member's Pharmacy Benefit (if one exists), even if the member meets the Medically Necessary criteria outlined below. There may also be circumstances when coagulation factor products need to be administered in the professional provider’s office when the member obtains the coagulation factor products from their pharmacy. Individual benefits must be verified as coverage may vary by product and/or group. Providers should work directly with the member's Pharmacy Benefit Administrator on options to obtain a self-administered drug through specialty pharmacy distribution under the member's Pharmacy Benefit. For more information on self-administered coagulation factors, refer to the Claim Payment Policy #08.00.78, Self-Administered Drugs (located in the Cross References section in this policy.)

When coagulation factors​ are administered by a professional provider in the outpatient facility, inpatient facility, or emergency room settings, coagulation factors may be eligible for coverage under the Medical Benefit when the medical necessity criteria are met.

US FOOD AND DRUG ADMINISTRATION (FDA) STATUS

There are numerous drugs approved by the FDA for the prevention and treatment of bleeding due to a factor deficiency.

Description

​​Hemophilia and von Willebrand disease (VWD) are the most common congenital bleeding disorders. Bleeding disorders are a group of conditions that result when the blood cannot clot properly. In normal clotting, platelets stick together and form a plug at the site of vascular damage. Proteins in the blood called coagulation factors then interact in a series of physiological processes to form an insoluble fibrin clot that holds the platelets in place and allows healing to occur at the site of the injury while preventing blood from escaping the blood vessel. The coagulation factors are indicated by Roman numerals (e.g., VII, VIII, and IX).

HEMOPHILIA: TYPES A, B, C

Hemophilia is a rare, typically inherited, bleeding disorder that can range from mild to severe, depending on how much clotting factor is present in the blood. Hemophilia A is considered the classic form of the disease, and is a consequence of a congenital deficiency of factor VIII. Hemophilia B, also called Christmas disease, is the result of a congenital deficiency of factor IX. Hemophilia C, a rare form of hemophilia, is the result of a congenital deficiency of factor XI. The defect results in the insufficient generation of thrombin by factor VIIIa, factor IX, or factor XI complex by means of the intrinsic pathway of the coagulation cascade. Individuals with less than 1% normal factor are considered to have severe hemophilia. Individuals with 1% to 5% normal factor are considered to have moderately severe hemophilia marked by spontaneous bleeding from the oral mucosa, joint bleeding, and bleeding from minor trauma. Individuals with more that 5% but less than 40% normal factor are considered to have mild hemophilia. Clinical bleeding symptom criteria have also been used for hemophilia classification when individuals have bleeding symptoms different than what would be expected from the plasma coagulant levels.

Because blood does not clot properly without enough clotting factor, any cut or injury carries the risk of excessive bleeding. The hallmark of congenital hemophilia is hemorrhage into the joints, or hemarthrosis. Synovial cells in joints synthesize high levels of tissue factor inhibitor, which predisposes hemophilic joints to bleed. This bleeding leads to progressive inflammation, deterioration, and deformities of the joint. In addition, people with hemophilia may suffer from internal bleeding that can damage organs and tissues over time.

TREATMENT​
Treatment options include fresh frozen plasma and clotting factor products that are made from human blood products such as donated plasma, or synthetic (recombinant) products. The administration of antihemophilic factor products temporarily replaces the missing clotting factor to treat or prevent bleeding episodes. Human preparations are manufactured from human plasma obtained and screened from tested United States donors for use in the treatment of bleeding episodes. Improved screening techniques and specific viral-inactivation treatments have made these products safer. A major breakthrough has enabled scientists to create synthetic blood factors, called recombinants, in the laboratory by cloning the genes responsible for specific clotting factors. Today’s factor replacement therapies are much safer, reducing the risk of viral pathogens such as hepatitis B, hepatitis C, and HIV infection.

Hemophilia A (Factor VIII Deficiency)

Some examples of the human preparations of coagulation factor VIII for the treatment of hemophilia A include Alphanate, Koate-DVI, Hemofil M, Humate-P, and Wilate. Recombinant antihemophilic factor products for use in those with hemophilia A include Advate, Adynovate, Afstyla, Altuviiio, Eloctate, Esperoct, Jivi, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, and Xyntha/Xyntha Solofuse. Additionally, Alhemo, Hemlibra ​and Hympavzi are examples of humanized monoclonal antibodies. Qfitlia (fitusiran) is an antithrombin-directed small interfering ribonucleic acid (siRNA). These products have various US Food and Drug Administration (FDA)–labeled indications, depending on the specific product, which may include prevention and control of bleeding episodes; short-term routine prophylactic treatment to reduce or prevent spontaneous musculoskeletal bleeding episodes and the risk of joint damage; and/or prevention or control of bleeding in surgical procedures in certain individuals with hemophilia A.

Jivi is not indicated for use in previously untreated individuals, and it also is not indicated for use in children younger than 7 years of age due to a greater risk for hypersensitivity reactions and/or loss of efficacy.

Hemophilia B (Factor IX Deficiency)

For the treatment of hemophilia B, human preparations of coagulation factor IX include Alphanine SD and Profilnine. Recombinant antihemophilic factor IX products include Alprolix, BeneFIX, Idelvion, Ixinity, Rebinyn, Rixubis. Additionally, Alhemo​ and Hympavzi are examples of humanized monoclonal antibodies. Fitusiran (Qfitlia) is an antithrombin-directed siRNA. These products have various FDA-labeled indications, depending on the specific product, which may include the prevention or control of bleeding during surgical procedures. In addition to those indications, Alprolix, Rebinyn, and Rixubis also have an FDA approval for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in individuals with hemophilia B.


Hemophilia C (Factor XI Deficiency)

For the treatment of hemophilia C, replacement therapy via factor XI concentrates are not currently available in the United States.

HEMOPHILIA WITH INHIBITORS TO FACTORS VIII OR IX

Some individuals with severe forms of hemophilia A or hemophilia B develop autoantibody inhibitors that can neutralize factor VIII or factor IX, respectively. They neutralize the coagulant effects of replacement therapy. The levels of factor inhibitors are often measured by using the Bethesda method. The Bethesda method quantitates the inhibitor titer.

TREATMENT
Treatment of hemophilia with inhibitors to factors VIII or IX is complex and ranges from products that increase factor levels to factor-bypassing agents. Several different immune tolerance therapy regimens have been developed. For example, if attempts to lower antibody levels with immunosuppressants or corticosteroids have been unsuccessful, large doses of replacement factor VIII or factor IX can be used to try to produce inhibitor suppression, or eradication can be utilized. In addition, genetically engineered or recombinant antihemophilic factor VIII (e.g., Advate, Kogenate FS, Recombinate) has the same biological effects as human factor VIII and has FDA-approved indications for certain individuals with factor VIII inhibitor disorder with inhibitor titers less than 10 Bethesda units per milliliter.

Additionally, FEIBA is a human plasma–derived activated prothrombin complex concentrate (APCC); its factor VIII inhibitor–​bypassing activity controls spontaneous bleeding episodes and is also used during surgical interventions in individuals who have hemophilia A and hemophilia B with inhibitors. In December 2013, FEIBA received FDA approval for use as routine prophylaxis to prevent or reduce the frequency of bleeding episodes in individuals with hemophilia A or B who have developed inhibitors.

Recombinant activated coagulation factor VIIa (e.g., NovoSeven RT, Sevenfact) has FDA-labeled indications for use in the treatment of bleeding episodes or for the prevention or control of bleeding in surgical interventions and invasive procedures in certain individuals with hemophilia who have inhibitors to factor VIII or factor IX. Emicizumab-kxwh (Hemlibra) is a human monoclonal antibody that was approved by the FDA for the treatment of hemophilia A (congenital factor VIII deficiency) with ​or without factor VIII inhibitors. Concizumab-mtci (Alhemo) is a human monoclonal antibody that was approved by the FDA for the treatment of hemophilia A or B with ​or without factor VIII or IX inhibitors​. Fitusiran (Qfitlia​) is an antithrombin-directed siRNA that was approved by the FDA for the treatment of hemophilia A or B with ​or without factor VIII or IX inhibitors.

FACTOR I DEFICIENCY, ACQUIRED 

Fibrinogen is a soluble protein in the bloodstream that is required to form clots. Fibrinogen is broken down to fibrin by the enzyme thrombin. Acquired fibrinogen deficiency (AFD)​ may be caused by hypofibrinogenemia (e.g., liver disease, plasma exchange therapy, disseminated intravascular coagulation [DІС], hemophagocytic lymphohistiocytosis [HLH]), inflammation, malignancy, or certain medications (e.g., fibrinolytic agents, isotretinoin, valproic acid). AFD is more common than congenital because liver disease and DIC are common. Individuals with AFD can be asymptomatic or present with either bleeding and/or thrombotic events.

TREATMENT
Fibryga (fibrinogen concentrate [Human]) was approved by the FDA for fibrinogen supplementation in bleeding individuals with AFD. In 2019, Callum et al. found that Fibryga (fibrinogen concentrate [Human]) was noninferior to cryoprecipitate with regard to number of blood components transfused in a 24-hour period after cardiac bypass surgery. 

FACTOR I DEFICIENCY, CONGENITAL

Fibrinogen is a soluble protein in the bloodstream that is required to form clots. Fibrinogen is broken down to fibrin by the enzyme thrombin. Factor I (fibrinogen) deficiency is a rare inherited bleeding disorder that comprises an absence of fibrinogen (afibrinogenemia), a low level of fibrinogen (hypofibrinogenemia), or fibrinogen of low quality that does not clot properly (dysfibrinogenemia).

TREATMENT
Management of acute hemorrhage includes cryoprecipitate or fresh frozen plasma. Additionally, there are FDA-approved fibrinogen concentrates (Human), Fibryga and RiaSTAP, indicated for the treatment of acute bleeding episodes in individuals with congenital fibrinogen deficiency, including afibrinogenemia and hypofibrinogenemia. (Note: Fibryga and RiaSTAP are not indicated for dysfibrinogenemia.)

FACTOR VII DEFICIENCY, CONGENITAL

Factor VII is one of the vitamin K–dependent coagulation factors synthesized in the liver. Plasma factor VII predominately exists in an inactive form; however, approximately 1% circulates in the activated form as factor VIIa. In response to injury or inflammation, factor VIIa activates other clotting factors, initiating the coagulation cascade. Congenital factor VII deficiency is a rare hemorrhagic disorder. The severity of the bleeding manifestations in affected individuals varies from mild to severe.

TREATMENT
Management of acute hemorrhage primarily consists of administration of factor VII to treat bleeding episodes. Because of the short half-life of factor VII, a treatment alternative may include recombinant-activated coagulation factor VIIa (NovoSevenRT), which is similar to human plasma–derived factor VIIa, with FDA-labeled indications that include the treatment of bleeding episodes and the prevention or control of bleeding in surgical interventions or invasive procedures in certain individuals who have a factor VII deficiency. The need for prophylaxis is determined by the individual's clinical presentation and the number of clinically significant bleeding episodes requiring intervention.

FACTOR VIII DEFICIENCY, ACQUIRED​

Acquired hemophilia A is the development of factor VIII inhibitors (autoantibodies) in persons without a history of factor VIII deficiency. It develops with a frequency of one case per one million population per year. Although the disorder is rare, it is known to cause significant morbidity and mortality. It may be associated with other disease cond​itions (e.g., collagen vascular disease, drug reaction, lymphoproliferative malignancies), or it can be idiopathic.

TREATMENT
Early treatment is directed toward achieving hemostasis and inhibitor eradication. Appropriate options for the treatment of bleeding episodes or for the prevention or control of bleeding in surgical procedures will depend on clinical presentation, and may include the use of immunosuppressive agents, desmopressin (1-desamino-8-D-arginine vasopressin [DDAVP]), Obizur and other factor VIII concentrates, antihemophilic factor VIII/von Willebrand factor complex (human) (Alphanate), recombinant-activated coagulation factor VIIa (NovoSevenRT) or FEIBA.

FACTOR VIII DEFICIENCY, CONGENITAL

See HEMOPHILIA A section (above).

FACTOR IX DEFICIENCY

See HEMOPHILIA B section (above).

FACTOR X DEFICIENCY, ACQUIRED OR CONGENITAL

Factor X is a vitamin K–dependent factor that is the first step in the common pathway to thrombus formation. Factor X can be activated by either the intrinsic or extrinsic clotting cascades. Factor X clotting deficiency may be acquired or congenital.

TREATMENT
Treatment options include fresh frozen plasma or plasma-derived prothrombin complex concentrates (plasma products containing a combination of vitamin K–dependent proteins) to stop or prevent bleeding. Additionally, Coagadex is the first human factor X concentrate approved by the FDA for the treatment of hereditary factor X deficiency. It is indicated for routine prophylaxis to reduce the frequency of bleeding episodes, on-demand treatment and control of bleeding episodes, and for perioperative (period extending from the time of hospitalization for surgery to the time of discharge) management of bleeding in individuals with mild, moderate, ​and severe hereditary factor X deficiency.

FACTOR XI DEFICIENCY, ACQUIRED OR CONGENITAL

See HEMOPHILIA C section (above).

FACTOR XIII DEFICIENCY

ACQUIRED FACTOR XIII DEFICIENCY
Acquired factor XIII deficiency has been described in association with a variety of diseases such as hepatic failure, inflammatory bowel disease, and myeloid leukemia. Deficiency of factor XIII can be corrected with infusions of fresh frozen plasma, cryoprecipitate, or factor XIII concentrates.

CONGENITAL FACTOR XIII DEFICIENCY
Factor XIII is the protein responsible for stabilizing the formation of a blood clot. Congenital factor XIII deficiency is known as fibrin-stabilizing factor deficiency. In the absence of factor XIII, a clot will still develop, but it will remain unstable. This condition, a rare autosomal disease, is perhaps the rarest of all factor deficiencies. It is a potentially life-threatening bleeding disorder in which the blood clots normally but the clots formed are unstable, leading to recurrent bleeding. It is estimated that the condition affects one in every three million to five million births. It affects men and women equally. Most individuals with factor XIII deficiency experience symptoms from birth, often bleeding from the umbilical cord stump. Symptoms such as menorrhagia, bleeding in soft tissue, abnormal bleeding during or after injury or surgery, and hemarthrosis tend to continue throughout life but may be managed prophylactically.

Treatment

Treatment options for factor XIII deficiency include cryoprecipitate or fresh frozen plasma, which are made from human blood products such as donated plasma. In February 2011, factor XIII concentrate (Human) (Corifact) was approved by the FDA for routine prophylactic treatment of congenital factor XIII deficiency and was later indicated for the perioperative management of surgical bleeding in adult and pediatric individuals ​with congenital factor XIII deficiency. Additionally, in December 2013, the FDA approved the first recombinant product, Coagulation Factor XIII A-Subunit (Recombinant) (Tretten®), for use in the routine prevention of bleeding in those who have congenital Factor XIII A–subunit deficiency.

GLYCOPROTEIN COMPLEX IIb/IIIa DEFICIENCY

Glanzmann thrombasthenia is a rare inherited bleeding disorder caused by an abnormality in the glycoprotein complex IIb/IIIa. This disorder prevents platelets from initiating clot formation.

TREATMENT
Treatment options for Glanzmann thrombasthenia include blood platelet transfusions and recombinant-activated coagulation factor VIIa (NovoSeven RT). NovoSeven RT was approved by the FDA for the treatment of bleeding episodes and perioperative management in adults and children with Glanzmann thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets.

VON WILLEBRAND DISEASE (VWD)

VWD is a congenital condition that results when there is an abnormality, either quantitative or qualitative, of von Willebrand factor. von Willebrand factor is required for normal platelet adhesion: it functions in both primary (involving platelet adhesion) and secondary (involving factor VIII) hemostasis. In primary hemostasis, von Willebrand factor attaches onto the platelet surface and acts as an adhesive bridge between the platelets and the damaged subendothelium at the site of vascular injury. In secondary hemostasis, von Willebrand factor protects factor VIII from degradation and delivers it to the site of injury. Bleeding tendency is usually mild and is characterized by a tendency for easy bruising, frequent epistaxis, and menorrhagia. VWD is classified into three different types (types 1, 2, and 3), based on the levels of von Willebrand factor and factor VIII activity in the blood. Type 1 is the mildest and most common form; type 3 is the most severe and least common form.

TREATMENT
Desmopressin (DDAVP) is a synthetic vasopressin analogue that has become a mainstay of therapy for most individuals with mild VWD. At appropriate doses, desmopressin causes a two-fold to five-fold increase in plasma von Willebrand factor and factor VIII concentrations in individuals who are healthy and responsive. Desmopressin can be used to treat bleeding complications or to prepare individuals with VWD for surgery. In a subtype of VWD (type IIB), desmopressin may cause a paradoxical drop in the platelet count and should not be used in a therapeutic setting without prior testing to see how the individual responds.

The goal of therapy is to correct the defect in platelet adhesiveness (by raising the level of effective von Willebrand factor) and to correct the defect in blood coagulation (by raising the level of factor VIII). Products such as von Willebrand factor/coagulation factor VIII complex (human) (Wilate) and antihemophilic factor/von Willebrand factor complexes (human) (Alphanate and Humate-P) are developed from human plasma consisting of two different proteins (factor VIII and von Willebrand factor).

Wilate has been FDA-approved for the treatment of spontaneous or trauma-induced bleeding episodes in certain individuals with severe VWD, as well as individuals with mild or moderate VWD in whom desmopressin is known or suspected to be ineffective or contraindicated. Wilate is indicated for the perioperative management of bleeding. Additionally, Wilate is indicated for routine prophylaxis in children 6 years of age and older and adults with vWD to reduce the frequency of bleeding episodes.

Alphanate has been FDA approved for surgical and/or invasive procedures in individuals with VWD in whom desmopressin (DDAVP) is either ineffective or contraindicated. This product is not indicated for individuals with severe VWD (type 3) undergoing major surgery.

Humate-P has been approved by the FDA for the treatment of spontaneous and trauma-induced bleeding episodes and for the prevention of excessive bleeding related to surgery in adult and pediatric individuals with ​VWD. ​This includes individuals with severe VWD, as well as individuals with mild and moderate VWD for whom desmopressin is known or suspected to be ineffective. In addition, Humate-P is the first product specifically FDA-approved for individuals with severe VWD who are undergoing major surgery.

A synthetic (recombinant) product, Vonvendi, has been approved by the FDA for adult and pediatric individuals with VWD for on-demand treatment and control of bleeding episodes and perioperative management of bleeding. Additionally, it is FDA approved for routine prophylaxis to reduce the frequency of bleeding episodes in adult patients only.

OFF-LABEL INDICATIONS

There may be additional indications contained in the Policy section of this document due to evaluation of criteria highlighted in the Company’s off-label policy, and/or review of clinical guidelines issued by leading professional organizations and government entities.​

References

Acharya SS. Pediatric von Willebrand disease. [eMedicine Web site]. 10/17/2024. Available at: https://emedicine.medscape.com/article/959825-overview. Accessed August 14, 2025.​ ​​​

Adynovate. Antihemophilic Factor (Recombinant), PEGylated. Prescribing information. Lexington, MA: Baxalta US Inc. 08/2023. Available at: https://www.adynovate.com/. Accessed August 14, 2025.​​​​

Advate. Antihemophilic factor (recombinant), plasma/albumin free. Prescribing information. Lexington, MA: Baxalta US Inc. 03/2025. Available at: http://www.advate.com/. Accessed August 14, 2025.​

Afstyla. Coagulation factor VIII (recombinant) [package insert]. CSL Behring LLC: Kankakee, IL. 06/2023. Available at: http://www.afstyla.com/?gclid=CNKd1drzhs0CFVAvgQod6pUHJw. Accessed August 14, 2025.​​​

A​lhemo. concizumab-mtci. Prescribing information. 07/2025. Novo Nordisk Health Care AG: Plainsboro, NJ. Available at:​ Treatment for Hemophilia | Alhemo® (concizumab-mtci) injection 60 mg, 150 mg, 300 mg. Accessed August 15, 2025.​​​

Alphanate. Antihemophilic factor/von Willebrand factor complex (human). [package insert]. Grifols Biologicals Inc. Los Angeles, CA. 11/2022. Available at: https://www.alphanate.com/en/patients. Accessed August 15, 2025.​​

Alphanine SD. Coagulation Factor IX (Human). Prescribing information. 11/2022. Grifols Biologicals Inc. Los Angeles, CA. Available at: Product Information​. Accessed August 14, 2025. ​​

Alprolix [Coagulation Factor IX (Recombinant), Fc Fusion Protein]. Prescribing information. 05/2023. Bioverativ Therapeutics Inc.; Waltham, MA. Available at: http://www.alprolix.com/. Accessed August 14, 2025.​​​

Altuviiio [antihemophilic factor (recombinant) Fc-VWF-XTEN fusion protein-ehtl]. Prescribing information. 03/2025. Bioverativ Therapeutics Inc.; Waltham, MA. ​Available at: ALTUVIIIO® Homepage | Sanofi | for US HCPs. Accessed August 14, 2025.​​

American Hospital Formulary Services (AHFS) Drug Information 2025. Antihemophilic factor (recombinant), Antihemophilic factor (human), Factor VIIa (Recombinant), Factor IX (human and recombinant), Factor XIII. [Lexicomp Online Web site]. Available at: http://online.lexi.com/lco/action/home [via subscription only]. Accessed August 14, 2025.​​​
​
BeneFIX. Coagulation factor IX (recombinant). Prescribing information. Philadelphia: Wyeth Pharmaceuticals Inc.; 11/2022. Available at: https://www.benefix.com/. Accessed August 14, 2025.​​​​

Bebulin (Factor IX complex, human). Discontinued Product via Lexi-Drugs.​

Bérubé C. Factor XI (eleven) deficiency. 04/10/2025. [UpToDate Web Site]. Available at [via subscription only]: http://www.uptodate.com/contents/factor-xi-deficiency?source=search_result&search=hemophilia+c&selectedTitle=1~24. Accessed August 14, 2025.​​​

Callum J, Farkouh ME, Scales DC, et al; FIBRES Research Group. Effect of Fibrinogen Concentrate vs Cryoprecipitate on Blood Component Transfusion After Cardiac Surgery: The FIBRES Randomized Clinical Trial. JAMA. 2019;26:322(20):1966-1976. 

Casini A, Bérubé C. Disorders of fibrinogen. 06/10/2025. [UpToDate Web Site]. Available at [via subscription only]: Disorders of fibrinogen - UpToDate. Accessed August 14, 2025.​

Centers for Disease Control and Prevention (CDC). Hemophilia treatment centers. [CDC Web site]. Updated 05/15/2024. Available at: Hemophilia Treatment Centers (HTCs) | Hemophilia | CDC​. Accessed August 14, 2025.​​​

Centers for Medicare & Medicaid Services (CMS). Medicare Benefit Policy Manual. Chapter 15: Covered Medical and Other Health Services. [CMS Website]. 50.5.5 - Hemophilia Clotting Factors. (Rev. 13108; Issued: 04-11-25​). Available at: https://www.cms.gov/Regulations-and-Guidance/Guidance/Manuals/Internet-Only-Manuals-IOMs-Items/CMS012673.html. Accessed August 14, 2025.

Centers for Medicare & Medicaid Services (CMS). Medicare Benefit Policy Manual. Chapter 17: Drugs and Biologicals​​. [CMS Website]. 80.4 - Billing for Hemophilia Clotting Factors. (Rev. 1564, Issued: 07-25-08, Effective: 04-01-08, Implementation: 01-05-09). Available at: Medicare Claims Processing Manual​. Accessed August 15, 2025.​

Centers for Medicare & Medicaid Services (CMS). National Coverage Determination (NCD) for Anti-Inhibitor Coagulant Complex (AICC) (110.3). Effective date not posted. Available at: https://www.cms.gov/medicare-coverage-database/details/ncd-details.aspx?NCDId=150&ncdver=1&DocID=110.3&bc=gAAAAAgAAAAAAA==&. Accessed August 14, 2025.​​​

Coagadex. Coagulation Factor X (Human). Prescribing information. 04/2023. Available at: http://www.coagadex.com/. Accessed August 15, 2025.​​​

​Company Benefit Contracts. 

Corifact, Factor XIII Concentrate (Human). Prescribing information. 09/2020. CSL Behring LLC: Kankakee, IL. Available at: http://www.corifact.com/. Accessed August 14, 2025.​​​​

Drelich DA. Hemophilia A (Factor VIII Deficiency). 04/30/2025. Available at: http://emedicine.medscape.com/article/779322-overview. Accessed August 14, 2025.​​​ ​

Eloctate (Recombinant antihemophilic Factor, FcFusion Protein). Prescribing information. 05/2023. Bioverativ Therapeutics Inc.; Waltham, MA. Available at: https://www.eloctate.com/. Accessed August 14, 2025.​​

Elsevier’s Clinical Pharmacology Compendium. 2025. [Clinical Key Web site]. Available at: https://www.clinicalkey.com/pharmacology/ [via subscription only]. Accessed August 14, 2025.​​​

Esperoct (Antihemophilic factor, recombinant, glycopegylated-exei). Prescribing information and supplemental biologics license approval letter. Updated 07/2024. Available at: https://www.esperoct.com/. Accessed August 14, 2025.​​​

FEIBA (Anti-Inhibitor Coagulant Complex). Prescribing information. 12/2024. Baxter Healthcare Corporation. Lexington, MA. Available at: www.feiba.com. Accessed August 14, 2025.​

Fibryga®. Fibrinogen (Human). Prescribing information. 11/2024. Octapharma USA, Inc. Hoboken, NJ. Available at: https://www.fibrygausa.com/#hcp. Accessed August 14, 2025.​​

Glanzmann’s thrombasthenia. NORD: National Organization for Rare Disorders. Published 2023. Available at: http://rarediseases.org/rare-diseases/glanzmann-thrombasthenia/. Accessed August 14, 2025.​​​

Gomperts E. Recombinant B domain deleted porcine factor VIII for the treatment of bleeding episodes in adults with acquired hemophilia A. Expert Rev Hematol. 2015;8(4):427-432. (Hyate:C. Discontinued Product)

Gupta V. Hemophilia C. 03/08/2022. Available at: http://emedicine.medscape.com/article/955690-overview. Accessed August 14, 2025.​​​

Helixate FS. Discontinued Product via Lexi-Drugs.

Hemlibra®. emicizumab-kxwh. Prescribing information. 07/2025. Genentech, Inc.; San Francisco, CA. Available at: https://www.hemlibra.com/. Accessed August 14, 2025.​​​

Hemophil M (Antihemophilic Factor [Human] Factor VIII). Lexington, MA: Baxalta US Inc. Prescribing information. 02/2025. Available at: https://www.takeda.com/en-us/what-we-do/product-portfolio/#section-h. Accessed August 14, 2025.​​​​

Hoffman M, Konkle BA. Recombinant factor VIIa: Administration and adverse effects. [UpToDate Web site]. 01/14/2025. Available at: http://www.uptodate.com/contents/therapeutic-uses-of-recombinant-coagulation-factor-viia [via subscription only]. Accessed August 14, 2025.​​

Humate-P. Antihemophilic factor/von Willebrand factor complex [human]. [package insert]. CSL Behring LLC Kankakee, IL.; 06/2020. Available at: http://www.humate-p.com/. Accessed August 15, 2025.​​​​

Hympavzi​. marstacimab-hncq. Prescribing information. 10/2024. Pfizer, Inc.; New York, NY. Available at: Help Enhance Coagulation with HYMPAVZI (marstacimab-hncq) | Official HCP Site​. Accessed August 14, 2025.​

Idelvion. Coagulation factor IX (recombinant). Prescribing information. 06/2023. CSL Behring LLC Kankakee, IL. Available at: http://www.idelvion.com/. Accessed August 14, 2025.​​​​

Ixinity. Coagulation Factor IX (Recombinant). Prescribing information. 03/2024. Medexus Pharma, Inc.: Chicago, IL​. Available at: https://www.ixinity.com/. Accessed August 14, 2025.​​​

James P. Acquired hemophilia A (and other acquired coagulation factor inhibitors)​. 06/20/2025. [UpToDate Web Site]. Available at: https://www.uptodate.com/contents/acquired-inhibitors-of-coagulation?topicRef=1336&source=see_link#H30 [via subscription only]. Accessed August 14, 2025.​

Jivi. Antihemophilic factor (recombinant) PEGylated-aucl. Prescribing information. 05/2025. Bayer Healthcare LLC: Whippany, NJ. Available at: https://www.jivi-us.com/. Accessed August 14, 2025.​​

Koate-DVI. Antihemophilic Factor (Human), Factor VIII. Prescribing information. 08/13/2025. Available at: Antihemophilic Factor, AHF, Factor VIII Indications - Clinical Pharmacology​. Accessed August 14, 2025.

Kogenate FS. Antihemophilic factor (recombinant). ​Lexi-Drugs (08.15/2025), Clinical Pharmacology (08/13/2025). Available at: ​Antihemophilic Factor (Recombinant) (Lexi-Drugs) - Lexicomp and Antihemophilic Factor, AHF, Factor VIII How Supplied - Clinical Pharmacology (clinicalkey.com)​. Accessed August 15, 2025.​​​

Kovaltry. Antihemophilic factor (recombinant). Prescribing information. 12/2022. Available at: https://www.kovaltry-us.com/. Accessed August 14, 2025.​​​

Lexi-Drugs Compendium. 2025. [Lexicomp Online Web site]. Available at: http://online.lexi.com/lco/action/home [via subscription only]. Accessed August 14, 2025.​​​
​
Lewandowska M. Acute treatment of bleeding and surgery in hemophilia A and B​. 04/30/2024. [UpToDate Web Site]. ​Available at: Acute treatment of bleeding and surgery in hemophilia A and B - UpToDate [via subscription only]. Accessed August 14, 2025.​

Ma AD, Carrizosa D. Acquired factor VIII inhibitors: pathophysiology and treatment. Hematology Am Soc Hematol Educ Program. 2006:432-437.

Malec L. Inhibitors in hemophilia: Mechanisms, prevalence, diagnosis, and eradication. 05/01/2025. [UpToDate Web Site]. Available at [via subscription only]: http://www.uptodate.com/contents/factor-viii-and-factor-ix-inhibitors-in-patients-with-hemophilia. Accessed August 14, 2025.​

Mannucci PM. Rare inherited coagulation disorders. 07/31/2025. [UpToDate Web site]. Available at: http://www.uptodate.com/contents/rare-recessively-inherited-coagulation-disorders?source=search_result&search=Rare+(recessively+inherited)+coagulation+disorders&selectedTitle=2~150 [via subscription only]. Accessed August 14, 2025.​​​​

Merative​. Micromedex® 2.0. DrugDex. Antihemophilic Factor (various). [Micromedex Web site]. 2024. Available at: http://www.micromedexsolutions.com/micromedex2/librarian/ [via subscription only]. Accessed August 14, 2025.

Monoclate-P. Discontinued Product via Lexi-Drugs. 

Mononine®. Discontinued Product via Lexi-Drugs and via Pharmaceutical Company correspondence​. 10/14/2024. Available at: Factor IX (Human) (Lexi-Drugs) - UpToDate® Lexidrug™. Accessed August 14, 2025.​​

Namburi S. Acquired Hemophilia Treatment & Management. [eMedicine Web site]. Treatment. 03/17/2023. Available at: http://emedicine.medscape.com/article/211186-treatment#showall. Accessed August 14, 2025.​​​

NovoSeven. Discontinued Product (Lexi-Drugs and via Pharmaceutical Company correspondence​). ​​

NovoSevenRT. Coagulation factor VIIa (recombinant). Prescribing information. 07/2020. Novo Nordisk Inc. Plainsboro, NJ. Available at: http://www.novosevenrt.com/. Accessed August 14, 2025.​​​

National Hemophilia Foundation (NHF). Medical and Scientific Advisory Council (MASAC) recommendations concerning products licensed for the treatment of hemophilia and other bleeding disorders. [NHF Web site]. 04/27/2022. Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&conten​tid=693. Accessed August 14, 2025.​​​​

National Hemophilia Foundation (NHF). Types of bleeding disorders. [NHF Web site]. 2025. Available at: https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders. Accessed August 14, 2025.​​​

National Organization of Rare Diseases (NORD). Acquired hemophilia. Updated 2022. Available at: https://rarediseases.org/rare-diseases/acquired-hemophilia/. Accessed August 14, 2025.​​​

Novitas Solutions, Inc. Local Coverage Article: Billing and Coding: Hemophilia Factor Products (A56433) [Novitas Solutions, Inc. Medicare Services Web site]. Original: 04/25/2019. Revised: 10/01/2023. Available at: Article - Billing and Coding: Hemophilia Factor Products (A56433) (cms.gov). Accessed August 14, 2025.​​
​
Novoeight. Coagulation factor VIII (recombinant). Prescribing information. 07/2020. Novo Nordisk Inc: Plainsboro, NJ. Available at: https://www.novoeight.com/. Accessed August 14, 2025.​​​​

Nuwiq. Coagulation factor VIII (recombinant). Prescribing information. 12/2024. Octapharma USA Inc: Hoboken, NJ. Available at: Hemophilia A Treatment - NUWIQ®️ [Antihemophilic Factor (Recombinant)] (nuwiqusa.com). Accessed August 14, 2025.​​​

Obizur. Antihemophilic Factor (Recombinant), Porcine s​equence. Prescribing information. 12/2024. Lexington, MA: Baxalta US Inc. Available at: http://www.obizur.com/. Accessed August 14, 2025.​​​​

Peyvandi F. Epidemiology and treatment of congenital fibrinogen deficiency. Thromb Res. 2012;130(Suppl 2):S7-11.

Profilnine. Coagulation factor IX complex (human). Los Angeles, CA: Grifols Biologics. 11/2022.​ Lexi-Drugs, Clinical Pharmacology. Available at: Product Catalogue and Factor IX How Supplied - Clinical Pharmacology and Factor IX Complex (Human) [(Factors II, IX, X)] (Lexi-Drugs) - UpToDate® Lexidrug™​. Accessed August 15, 2025.

Profilnine SD. Coagulation factor IX complex (human). Discontinued Product in 2016 via Grifols correspondence, Lexi-Drugs, Micromedex. ​​

Qfitlia. (fitusiran). Prescribing information. 03/28/2025. Genzyme Corporation: Cambridge, MA​. Available at: QFITLIA® (fitusiran) | For US HCPs. Accessed August 15, 2025.​​​​

Rebinyn. Coagulation factor IX (recombinant) GlycoPEGylated). Prescribing information. 08/2022. Novo Nordisk Inc. Plainsboro, NJ. Available at: https://www.rebinyn.com/. Accessed August 14, 2025.​​​​

RECOMBINATE. Antihemophilic factor, recombinant. Prescribing information. 03/2025. Lexington, MA: Baxalta US Inc. Available at: http://www.recombinate.com/. Accessed August 14, 2025.​​​

Refacto®. Antihemophilic factor, recombinant. Discontinued Product via Lexi-Drugs and Clinical Pharmacology (08/13/2025). Available at: https://www.clinicalkey.com/pharmacology/monograph/40?sec=monsup&n=ReFacto. Accessed August 14, 2025.​​​​

RiaSTAP®. [Package Insert]. Labeling revised 06/2021. Kankakee, IL: CSL Behring LLC. Available at: http://www.riastap.com/. Accessed August 14, 2025.​​

Rixubis. Coagulation Factor IX (Recombinant). Prescribing information. 03/2025. Baxter US Inc: Lexington, MA. Available at: https://www.rixubis.com/. Accessed August 14, 2025.​​​​

Roberts HR, Hoffman M. Hemophilia A and hemophilia B. In: Butler E, Coller BS, Lichtman MA, Kipps TJ, Seligsohn U, eds. Hematology. 6th ed. New York, NY: McGraw-Hill; 2001:1639-57.

Rudisill CN, Hockman RH, Degregory KA, et al. Implementing guidelines for the institutional use of factor VIIa (recombinant): a multidisciplinary solution. Am J Health Syst Pharm. 2006;63(17):1641-1646.

​Schwartz RA. Factor X Deficiency. [eMedicine Web site]. 03/18/2024. Available at: http://emedicine.medscape.com/article/209867-overview. Accessed August 14, 2025.​​​​

Schwartz RA. Factor XIII Deficiency Treatment & Management. [eMedicine Web site]. 03/18/2024. Available at: http://emedicine.medscape.com/article/209179-treatment. Accessed November 1, 2024.​​

Sevenfact®. Coagulation factor VIIa (recombinant)-jncw. Prescribing information. 03/11/2025. HEMA Biologics, LLC: Louisville, KY. Available at: ​https://sevenfact.com/. Accessed August 14, 2025.​​

Tahlan A, Ahluwalia J. Factor XIII: congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit. Arch Pathol Lab Med. 2014;138(2):278-281.

Tretten. Coagulation Factor XIII A-Subunit (Recombinant). Prescribing information. 06/2020. Novo Nordisk Inc. Plainsboro, NJ. Available at: http://www.tretten.com/. Accessed August 14, 2025.​​​​

Vonvendi. von Willebrand Factor (Recombinant). Prescribing information. 09/2025. Baxalta US Inc: Lexington, MA. Available at: VONVENDI® [von Willebrand factor (Recombinant)] for HCPs. Accessed September 17, 2025.​​​

Wilate. von Willebrand factor/coagulation factor VIII complex [human]). Prescribing information. 12/2024. Octapharma USA, Inc.: Hoboken, NJ. Available at: https://www.wilateusa.com/. Accessed August 15, 2025.​​​​

Wilczynski C. Fibrinogen. [eMedicine Web site]. 11/21/2019. Available at: http://emedicine.medscape.com/article/2085501-overview#showall. Accessed August 14, 2025.​​

Xyntha/Xyntha Solofuse. Antihemophilic factor (recombinant). Product information. 07/2022. Available at: https://www.xyntha.com/. Accessed August 14, 2025.​ ​​

Zaiden RA. Hemophilia B (Factor IX Deficiency). [eMedicine Web site]. 04/30/2025. Available at:  http://emedicine.medscape.com/article/199088-medication#showall. Accessed August 14, 2025.​ ​​

Coding

CPT Procedure Code Number(s)
N/A
ICD - 10 Procedure Code Number(s)
N/A
ICD - 10 Diagnosis Code Number(s)
Report the most appropriate diagnosis code in support of medically necessary criteria as listed in the policy.​
HCPCS Level II Code Number(s)

MEDICALLY NECESSARY

J7170 Injection, emicizumab-kxwh, 0.5 mg

J7172 Injection, marstacimab-hncq, 0.5 mg

J7174 Injection, fitusiran, 0.04 mg

J7177 Injection, human fibrinogen concentrate (fibryga), 1 mg

J7179 Injection, von Willebrand factor (recombinant), (Vonvendi), 1 IU VWF:RCo

J7182 Injection, factor VIII, (antihemophilic factor, recombinant), (NovoEight), per IU

J7183 Injection, von Willebrand factor complex (human), Wilate, 1 IU vWF:RCo

J7185 Injection, factor VIII (antihemophilic factor, recombinant) (Xyntha), per IU

J7187 Injection, von Willebrand factor complex (Humate-P), per IU VWF:RCO

J7189 Factor viia (antihemophilic factor, recombinant), (novoseven rt), 1 microgram

J7200 Injection, factor IX, (antihemophilic factor, recombinant), Rixubis, per IU

J7201 Injection, factor IX, Fc fusion protein, (recombinant), Alprolix, 1 IU

J7202 Injection, factor IX, albumin fusion protein, (recombinant), Idelvion, 1 IU

J7203 Injection factor ix, (antihemophilic factor, recombinant), glycopegylated, (rebinyn), 1 iu

J7204 Injection, factor viii, antihemophilic factor (recombinant), (esperoct), glycopegylated-exei, per iu

J7208 Injection, factor viii, (antihemophilic factor, recombinant), pegylated-aucl, (jivi), 1 i.u.

J7209 Injection, factor VIII, (antihemophilic factor, recombinant), (Nuwiq), 1 IU

J7210 Injection, factor viii, (antihemophilic factor, recombinant), (Afstyla), 1 I.U.

J7211 Injection, factor viii, (antihemophilic factor, recombinant), (Kovaltry), 1 I.U.

J7212 Factor viia (antihemophilic factor, recombinant)-jncw (sevenfact), 1 microgram

J7213 Injection, coagulation factor ix (recombinant), ixinity, 1 i.u.

J7214 Injection, Factor VIII/von Willebrand factor complex, recombinant (Altuviiio), per Factor VIII IU


THE FOLLOWING CODE IS USED TO REPRESENT Coagadex®:

J7175 Injection, factor X, (human), 1 IU


THE FOLLOWING CODE IS USED TO REPRESENT RiaSTAP®:

J7178 Injection, human fibrinogen concentrate, not otherwise specified, 1 mg


THE FOLLOWING CODE IS USED TO REPRESENT Corifact®:

J7180 Injection, factor XIII (antihemophilic factor, human), 1 IU


THE FOLLOWING CODE IS USED TO REPRESENT Tretten®:

J7181 Injection, factor XIII A-subunit, (recombinant), per IU


THE FOLLOWING CODE IS USED TO REPRESENT Alphanate®:

J7186 Injection, antihemophilic factor VIII/von Willebrand factor complex (human), per factor VIII i.u.


THE FOLLOWING CODE IS USED TO REPRESENT Obizur:

J7188 Injection, factor VIII (antihemophilic factor, recombinant), per IU


THE FOLLOWING CODE IS USED TO REPRESENT Koate®DVI and Hemofil M:

J7190 Factor VIII (antihemophilic factor, human) per IU


THE FOLLOWING CODE IS USED TO REPRESENT Advate®, Kogenate FS®, and Recombinate®:

J7192 Factor VIII (antihemophilic factor, recombinant) per IU, not otherwise specified


THE FOLLOWING CODE IS USED TO REPRESENT Alphanine® SD:

J7193 Factor IX (antihemophilic factor, purified, nonrecombinant) per IU


THE FOLLOWING CODE IS USED TO REPRESENT Profilnine®:

J7194 Factor IX complex, per IU


THE FOLLOWING CODE IS USED TO REPRESENT BeneFIX®:

J7195 Injection, factor IX (antihemophilic factor, recombinant) per IU, not otherwise specified


THE FOLLOWING CODE IS USED TO REPRESENT FEIBA (human plasma-derived activated prothrombin complex concentrate [APCC]):

J7198 Antiinhibitor, per IU


THE FOLLOWING CODE IS USED TO REPRESENT Eloctate®:

J7205 Injection, factor VIII Fc fusion protein (recombinant), per IU


THE FOLLOWING CODE IS USED TO REPRESENT Adynovate:

J7207 Injection, factor VIII, (antihemophilic factor, recombinant), PEGylated, 1 IU


THE FOLLOWING CODES ARE USED TO REPRESENT Concizumab-mtci (Alhemo):

C9399 Unclassified drugs or biologicals

J3590 Unclassified biologics


NOT ELIGIBLE FOR REIMBURSEMENT


THE FOLLOWING CODES REPRESENT BEBULIN, HELIXATE® FS, HYATE:C®, MONOCLATE-P®, MONONINE®, NOVOSEVEN®, PROFILNINE®SD, AND REFACTO® WHICH ARE NO LONGER MANUFACTURED AND HAVE BEEN WITHDRAWN FROM THE MARKET:


J7189 Factor VIIa (atihemophilic factor, recombinant), per 1 mcg

J7190 Factor VIII (antihemophilic factor, human) per IU

J7191 Factor VIII (antihemophilic factor (porcine), per IU

J7192 Factor VIII (antihemophilic factor, recombinant) per IU, not otherwise specified

J7193 Factor IX (antihemophilic factor, purified, nonrecombinant) per IU

J7194 Factor IX complex, per IU

Revenue Code Number(s)
N/A

​

Coding and Billing Requirements

BILLING REQUIREMENTS

If there is no specific HCPCS code available for the drug administered, then the drug must be reported with the most appropriate unlisted code along with the corresponding National Drug Code (NDC).

Policy History

Revisions From 08.00.92ak:​
01/01/2026​
This version of the policy will become effective 01/01/2026​.
​
This policy has been updated to communicate the coverage position for the following two new products: ​

  • Concizumab-mtci (Alhemo). Monoclonal antibody for the treatment of hemophilia A and B with or without FVIII and FIX inhibitors. 

  • Fitusiran (Qfitlia). Small interfering ribonucleic acid for the treatment of hemophilia A or B with or without factor VIII or IX inhibitors.​

The disclaimer for Pennsylvania Products (Benefits) was moved from the Policy Section into the Guidelines Section.  


The following HCPCS code has been added to this policy for fitusiran (Qfitlia):

J7174 Injection fitusiran 0.04 mg​


THE FOLLOWING CODES ARE USED TO REPRESENT Concizumab-mtci (Alhemo):
C9399 Unclassified drugs or biologicals
J3590 Unclassified biologics
​
All of the ICD-10 CM codes have been removed from this policy. Report the most appropriate diagnosis code in support of medically necessary criteria as listed in the policy.​

Revisions From 08.00.92aj:​
07/01/2025
This version of the policy will become effective 07/01/2025.

The following HCPCS code has been added to this policy:
J7172 Injection, marstacimab-hncq, 0.5 mg

The following HCPCS codes have been removed from this policy for marstacimab-hncq (Hympavzi): 
C9399 Unclassified drugs or biologicals
J3590  Unclassified biologics

Revisions From 08.00.92ai:
04/01/2025
This version of the policy will become effective 04/01/2025.

The following HCPCS code has been added to this policy:
C9304 Injection, marstacimab-hncq, 0.5 mg

The following HCPCS code has been removed from this policy for marstacimab-hncq
(Hympavzi): C9399 Unclassified drugs or biologics​

Revisions From 08.00.92ah:
01/06/2025
This version of the policy will become effective 01/06/2025​.​

This policy has been updated to communicate the coverage position for the following, in consideration of revisions within the US Food and Drug Administration (FDA) labeling:
  • The new product, marstacimab-hncq (Hympavzi​) was added to this policy for the treatment of hemophilia A and B. 
  • Mononine has been discontinued by the pharmaceutical company.
  • Fibryga (fibrinogen concentrate [Human]) is covered for the treatment of bleeding episodes in individuals with​ acquired fibrinogen deficiency (AFD).
  • Coagadex is covered for the prevention and/or treatment of bleeding episodes in individuals with mild, moderate, or severe factor X deficiency.
  • Wilate is covered for routine prophylaxis in children 6 years of age and older and adults with von Willebrand disease to reduce the frequency of bleeding episodes.
The following changes to coding have been made:
  • All of the ICD-10 CM codes have been removed from this policy, because they are informational.​
  • The following HCPCS codes have been added to this policy for marstacimab-hncq (Hympavzi):

C9399 Unclassified drugs or biologicals

J3590  Unclassified biologics


  • ​Mononine was discontinued. HCPCS code "J7193 Factor IX (antihemophilic factor, purified, nonrecombinant) per IU" will be considered Not Eligible for Reimbursement.​

Revisions From 08.00.92ag:
01/02/2024 This version of the policy will become effective 01/02/2024​​.

This policy was updated to communicate the coverage position for the following:
  • New Jersey Products: Coagulation factors​ are available under the Medical Benefit.
  • Altuviiio, represented by HCPCS ​​J7214,​​ for the treatment for Hemophilia A.​

Revisions From 08.00.92af:
07/01/2023This version of the policy will become effective 07/01/2023.

The following HCPCS code has been replaced by the following code for Ixinity. (Ixinity will no longer be represented by J7195, but will remain in the policy for Benefix.):

Removed: J7195 Injection, factor IX (antihemophilic factor, recombinant) per IU, not otherwise specified
Replaced With: J7213 Injection, coagulation factor ix (recombinant), ixinity, 1 i.u.

Revisions From ​08.00.92ae:
11/02/2022
​​The policy has been reviewed and reissued to communicate the Company’s continuing position on Coagulation Factors.​

This policy has been updated in consideration of revisions within the US Food and Drug Administration (FDA) labeling:
  • The new product, Xyntha Solofuse, was added to this policy for the treatment of hemophilia A. It is represented by HCPCS J7185, which already exists in the policy. 
  • ​Rebinyn received a supplemental FDA-approval for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in individuals with hemophilia B.​
10/01/2022

This version of the policy will become effective 10/01/2022.

​The following ICD-10 CM code has been termed (no longer valid codes) and removed from this policy:
D68.0 Von Willebrand's disease

The following ICD-10 CM codes have been added to this policy:
D68.00 Von Willebrand disease, unspecified
D68.01 Von Willebrand disease, type 1
D68.020 Von Willebrand disease, type 2A
D68.021 Von Willebrand disease, type 2B
D68.022 Von Willebrand disease, type 2M
D68.023 Von Willebrand disease, type 2N
D68.029 Von Willebrand disease, type 2, unspecified
D68.03 Von Willebrand disease, type 3
D68.04 Acquired von Willebrand disease
D68.09 Other von Willebrand disease

Revisions From 08.00.92ad:
10/25/2021This version of the policy will become effective 10/25/2021​​.

This policy was updated to clarify the coverage position for the following:
  • Hemophil M was ​misspelled. Revised to be Hemofil M.
  • Profilnine was added to the policy as Medically Necessary. HCPCS code J7194 Factor IX complex, per IU​
  • Profilnine SD was discontinued. HCPCS code J7194 Factor IX complex, per IU will be considered Not Eligible for Reimbursement.

Revisions From 08.00.92ac:
01/01/2021This policy was updated to communicate the coverage position for the following:
  • New FDA-approved product: Name changed from Eptacog Beta to Sevenfact® (HCPCS J7189).
  • New indication: Wilate® in individuals with hemophilia A
  • Products discontinued by the Pharmaceutical Companies and are not eligible for reimbursement: Helixate® FS (HCPCS J7192), Monoclate-P® (HCPCS J7190), NovoSeven® (HCPCS J7189), ReFacto® (HCPCS J7192)
  • Coagadex® updated per FDA - not indicated for perioperative in severe disease.
  • Categorization of Hemlibra® changed from a recombinant preparation for hemophilia to a human monoclonal antibody for hemophilia A.
  • Tretten® expanded for use in those with acquired or congenital factor XIII deficiency
  • Afstyla HCPCS code change from J7192 to J7210.
The effective date of this policy has been revised from 12/28/2020 to 01/01/2021, to include to quarterly code updates.

The following HCPCS narrative has been revised in this policy:

FROM: J7189 Factor VIIa (antihemophilic factor, recombinant), per 1 mcg
TO: J7189 Factor viia (antihemophilic factor, recombinant), (novoseven rt), 1 microgram

 

The following HCPCS code has been removed for Sevenfact and is replaced by the following HCPCS code:

REMOVED: J7189 Factor VIIa (antihemophilic factor, recombinant), per 1 mcg

REPLACED WITH: J7212 Factor viia (antihemophilic factor, recombinant)-jncw (sevenfact), 1 microgram​​


Revisions From 08.00.92ab:
07/01/2020This policy has been identified for the HCPCS code update, effective 07/01/2020.

The following code has been removed from this policy to represent Esperoct, and is replaced by the following HCPCS code:

REMOVED: J7192 Factor VIII (antihemophilic factor, recombinant) per IU, not otherwise specified
REPLACED WITH: J7204 Injection, factor viii, antihemophilic factor (recombinant), (esperoct), glycopegylated-exei, per iu

Revisions From 08.00.92aa:
10/21/2019This policy has been updated to communicate the coverage criteria for two products:
  • Medically Necessary coverage criteria has been added for a new hemophilia A recombinant product, Esperoct
  • Bebulin changed from Medically Necessary to Not Eligible For Reimbursement, since withdrawn from the market
Additionally, ICD-10 D68.1 Hereditary factor XI deficiency has been removed, since there are no FDA-approved products for hemophilia C.

Revisions From 08.00.92z:
07/01/2019This policy has been identified for the HCPCS code update, effective 07/01/2019.

The following HCPCS code has been added to this policy as Medically Necessary:
J7208 Injection, factor viii, (antihemophilic factor, recombinant), pegylated-aucl, (jivi), 1 i.u.
  • Note: This code no longer represents Jivi: J7207 Injection, factor VIII, (antihemophilic factor, recombinant), PEGylated, 1 IU

The following HCPCS code has been removed from this policy:
C9141 Injection, factor viii, (antihemophilic factor, recombinant), pegylated-aucl (jivi), 1 i.u

Revisions From 08.00.92y:
04/01/2019This policy has been identified for the HCPCS code update, effective 04/01/2019.

The following HCPCS code has been added to this policy as Medically Necessary:

C9141 Injection, factor viii, (antihemophilic factor, recombinant), pegylated-aucl (jivi), 1 i.u.

Revisions From 08.00.92x:
01/01/2019This policy has been updated in consideration of revisions within the US Food and Drug Administration (FDA) labeling.
  • Jivi®, a recombinant DNA-derived Factor VIII product, has been added to this policy as Medically Necessary.
  • The coverage of Hemlibra® has been updated to include the new indication of hemophilia A (congenital factor VIII deficiency) with or without factor VIII inhibitors.
  • There has been a brand name change: FROM: Fibryna TO: Fibryga

The following HCPCS codes have been added/revised/deleted:
  • Fibryga HCPCS Code Change:
    • FROM: J7178 Injection, human fibrinogen concentrate, 1 mg
    • TO: J7177 Injection, human fibrinogen concentrate (fibryga), 1 mg
  • Riastap HCPCS Narrative Change:
    • FROM: J7178 Injection, human fibrinogen concentrate, 1 mg
    • TO: J7178 Injection, human fibrinogen concentrate, not otherwise specified, 1 mg
  • Rebinyn HCPCS coding changes
    • Delete: C9468 Injection, factor ix (antihemophilic factor, recombinant), glycopegylated, Rebinyn, 1 i.u.
    • Update:
      • FROM: J7195 Injection, factor IX (antihemophilic factor, recombinant) per IU, not otherwise specified
      • TO: J7203 Injection factor ix, (antihemophilic factor, recombinant), glycopegylated, (rebinyn), 1 iu
  • Hemlibra HCPCS coding changes
    • FROM: Q9995 Injection, emicizumab-kxwh, 0.5 mg
    • TO: J7170 Injection, emicizumab-kxwh, 0.5 mg

Revisions From 08.00.92w:
07/01/2018This policy has been identified for the HCPCS code update, effective 04/01/2018.

The following HCPCS code has been added and the corresponding non-specific code removed from this policy:

ADDED: Q9995 Injection, emicizumab-kxwh, 0.5 mg
REMOVED: J7199 Hemophilia clotting factor, not otherwise classified

Revisions From 08.00.92v:
05/28/2018This policy has been updated in consideration of revisions within the US Food and Drug Administration (FDA) labeling.

Eptacog Beta and emicizumab-kxwh (Hemlibra®) have been added to this policy as Medically Necessary. Fibryna® and RiaSTAP® have been updated as experimental/investigational for the treatment of dysfibrinogenemia.

Revisions From 08.00.92u:
04/01/2018This policy has been identified for the HCPCS code update, effective 04/01/2018.

The following HCPCS code has been added to this policy:
C9468 Injection, factor ix (antihemophilic factor, recombinant), glycopegylated, Rebinyn, 1 i.u.

Revisions From 08.00.92t:
01/01/2018This policy has been identified for the HCPCS code update, effective 01/01/2018.

The following HCPCS code has been removed from this policy:
C9140 Injection, factor VIII (antihemophilic factor, recombinant) (Afstyla), 1 i.u.

The following ICD-10 CM codes have been added to this policy:
J7210 Injection, factor viii, (antihemophilic factor, recombinant), (Afstyla), 1 i.u.
J7211 Injection, factor viii, (antihemophilic factor, recombinant), (Kovaltry), 1 i.u.

Effective 10/05/2017 this policy has been updated to the new policy template format.​
1/1/2026
1/2/2026
08.00.92
Medical Policy Bulletin
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