Notification



Notification Issue Date:



Medical Policy Bulletin


Title:Coagulation Factors

Policy #:08.00.92aa

This policy is applicable to the Company’s commercial products only. Policies that are applicable to the Company’s Medicare Advantage products are accessible via a separate Medicare Advantage policy database.


The Company makes decisions on coverage based on Policy Bulletins, benefit plan documents, and the member’s medical history and condition. Benefits may vary based on contract, and individual member benefits must be verified. The Company determines medical necessity only if the benefit exists and no contract exclusions are applicable.

When services can be administered in various settings, 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. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.

This Medical Policy Bulletin document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy Bulletin will be reviewed regularly and be updated as scientific and medical literature becomes available. For more information on how Medical Policy Bulletins are developed, go to the About This Site section of this Medical Policy Web site.



Policy

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

When services can be administered in various settings, 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. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.

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

FOR MEMBERS ENROLLED IN NEW JERSEY PRODUCTS

When the medical necessity criteria listed below are met, coagulation factors may be available under the applicable Medical or Pharmacy benefit. Individual benefits must be verified.

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, Hemophil M, Humate-P®, and Monoclate-P®.
  • recombinant preparations: Advate®, Adynovate, Afstyla®, Eloctate, Esperoct, Helixate® FS, Hemlibra®, Jivi®, Kogenate FS®, Kovaltry, Novoeight®, Nuwiq®, Recombinate®, ReFacto®, and Xyntha®

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, Mononine®, and Profilnine® SD
  • recombinant preparations: Alprolix™, BeneFIX®, Idelvion®, Ixinity®, Rebinyn®, and Rixubis®

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., Eptacog Beta, NovoSeven®, NovoSevenRT®)
  • human monoclonal antibody (e.g., emicizumab-kxwh [Hemlibra®]) for hemophilia with inhibitors to factor VIII

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® and NovoSevenRT® (recombinant-activated coagulation factor VIIa) are 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®, NovoSevenRT® (recombinant-activated coagulation factor VIIa)
  • FEIBA (human plasma-derived activated prothrombin complex concentrate [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]) for use in those with congenital Factor XIII A-subunit deficiency

GLANZMANN'S THROMBASTHENIA (GLYCOPROTEIN COMPLEX IIb/IIIa DEFICIENCY)
NovoSevenRT® (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’s 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 FDA 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 and Hyate:C® 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.

Subject to the terms and conditions of the applicable benefit contract, for members enrolled in Pennsylvania Products, coagulation factors are generally not covered under the medical benefits of the Company’s products, even when the medical necessity criteria listed in this medical policy are met.

Subject to the terms and conditions of the applicable benefit contract, for members enrolled in New Jersey Products, coagulation factors are covered under the medical benefits of the Company’s products when the medical necessity criteria listed in this medical policy 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 percent normal factor are considered to have severe hemophilia. Individuals with 1 percent to 5 percent 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 percent but less than 40 percent 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, Hemophil M, Humate-P®, and Monoclate-P®. Recombinant antihemophilic factor products for use in those with hemophilia A include Advate®, Adynovate, Afstyla®, Eloctate, Esperoct, Helixate® FS, Hemlibra®, Jivi®, Kogenate FS®, Kovaltry, Novoeight®, Nuwiq®, Recombinate®, ReFacto®, and Xyntha®. 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 not indicated for use in children less than 12 years of age due to a greater risk for hypersensitivity reactions.

Hemophilia B (Factor IX Deficiency)

For the treatment of hemophilia B, human preparations of coagulation factor IX includes Alphanine® SD, Mononine®, and Profilnine® SD. Recombinant antihemophilic factor IX products include Alprolix™, BeneFIX®, Idelvion®, Ixinity®, Rebinyn®, Rixubis®. These products have various US Food and Drug Administration (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™ 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®, Helixate® FS, Kogenate FS®, Recombinate®, ReFacto®) 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) whose 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 patients with hemophilia A or B who have developed inhibitors.

Recombinant activated coagulation factor VIIa (e.g., Eptacog Beta, NovoSeven®, NovoSevenRT®) 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 factor VIII inhibitors.

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 is comprised of 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 include 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 one percent 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. Due to the short half-life of factor VII, a treatment alternative may include recombinant-activated coagulation factor VIIa (NovoSeven®, 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. Though the disorder is rare, it is known to cause significant morbidity and mortality. It may be associated with other disease conditions (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 (NovoSeven®, 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 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 patients with mild 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 peri-operative management of surgical bleeding in adult and pediatric patients with congenital factor XIII deficiency. Corifact™ received orphan drug designation by the FDA because it is intended for use in a rare disease or condition. It was approved for marketing under the FDA's accelerated approval regulations. Additionally, in December 2013, the FDA approved the first recombinant product, 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’s 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’s thrombasthenia include blood platelet transfusions and recombinant-activated coagulation factor VIIa (NovoSevenRT®). NovoSevenRT® was approved by the FDA for the treatment of bleeding episodes and peri-operative management in adults and children with Glanzmann’s thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets.

VON WILLEBRAND DISEASE (VWD)

Von Willebrand disease (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 (1-desamino-8-D-arginine vasopressin [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. This product is also indicated for the perioperative management of bleeding.

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 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. The FDA has also approved Humate-P® in 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.

Additionally, a synthetic (recombinant) product, Vonvendi, has been FDA approved for the on-demand treatment and control of bleeding episodes in adults diagnosed with von Willebrand disease. This product was later approved for the perioperative management of bleeding.

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 Willenbrand disease. [eMedicine Web site]. 02/05/2019. Available at: https://emedicine.medscape.com/article/959825-overview. Accessed July 22, 2019.


Adynovate. Antihemophilic Factor (Recombinant), PEGylated. Prescribing information. Lexington, MA: Baxalta US Inc. 05/2018. Available at: https://www.adynovate.com/ . Accessed July 19, 2019.

Advate®. Antihemophilic factor (recombinant), plasma/albumin free. Prescribing information. Lexington, MA: Baxalta US Inc. 12/2018. Available at: http://www.advate.com/ . Accessed July 19, 2019.

Afstyla®. Coagulation factor VIII (recombinant) [package insert]. CSL Behring LLC: Kankakee, IL. 09/2017. Available at: http://www.afstyla.com/?gclid=CNKd1drzhs0CFVAvgQod6pUHJw . Accessed July 19, 2019.

Alphanate®. Antihemophilic factor/von Willebrand factor complex (human). [package insert]. Grifols Biologicals Inc. Los Angeles, CA. 06/2018. Available at: https://www.alphanate.com/en/patients . Accessed July 19, 2019.

Alphanine® SD. Coagulation Factor IX (Human). Prescribing information. 06/2018. Grifols Biologicals Inc. Los Angeles, CA. Available at: http://www.alphaninesd.com/en/web/alphanine/home. Accessed July 22, 2019.

Alprolix™ [Coagulation Factor IX (Recombinant), Fc Fusion Protein]. Prescribing information. 06/2018. Bioverativ Therapeutics Inc.: Waltham, MA. Available at: http://www.alprolix.com/. Accessed July 22, 2019.

American Hospital Formulary Services (AHFS) Drug Information 2019. 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 July 22, 2019.

BeneFIX®. Coagulation factor IX (recombinant). Prescribing information. Philadelphia, PA: Wyeth Pharmaceuticals Inc. 06/2017. Available at: https://www.benefix.com/ . Accessed July 22, 2019.

Bolton-Maggs PHB. Mathew P. Hemophilia C. 03/01/2019. Available at: http://emedicine.medscape.com/article/955690-overview . Accessed July 22, 2019.

Bebulin (Factor IX complex, human). Baxalta US Inc. Westlake Village, CA. Prescribing information. 09/2015. Available at: https://www.hemophilia.org/Newsroom/Medical-News/Shire-Discontinuing-Manufacture-and-Distribution-of-BEBULIN andhttp://online.lexi.com/lco/action/doc/retrieve/docid/patch_f/6890?searchUrl=%2Flco%2Faction%2Fsearch%3Fq%3Dbebulin%26t%3Dname%26va%3Dbebulin and http://www.shirecontent.com/PI/PDFs/BEBULIN_USA_ENG.pdf . Accessed July 22, 2019.

Bérubé C. Factor XI deficiency. 06/11/19. [UpToDate Web Site]. Available at [via subscription only]: http://www.uptodate.com/contents/factor-xi-deficiency?source=search_result&search=hemophilia+c&selectedTitle=1%7E24 . Accessed July 22, 2019.

Center for Disease Control and Prevention (CDC). Hemophilia treatment centers. [CDC Web site]. Updated 09/06/18. Available at: http://www.cdc.gov/ncbddd/hemophilia/HTC.html. Accessed July 22, 2019.

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 1, 10/01/03). Available at: https://www.cms.gov/Regulations-and-Guidance/Guidance/Manuals/Internet-Only-Manuals-IOMs-Items/CMS012673.html. Accessed July 19, 2019.

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%3d%3d&. Accessed July 19, 2019.

Corifact, Factor XIII Concentrate (Human). Prescribing information. 09/2017. CSL Behring LLC: Kankakee, IL. Available at: http://www.corifact.com/ . Accessed July 22, 2019.

Drelich DA. Hemophilia A. 01/14/19. Available at: http://emedicine.medscape.com/article/779322-overview . Accessed July 22, 2019.

Eloctate (Recombinant antihemophilic Factor, FcFusion Protein). Prescribing information. 12/2017. Bioverativ Therapeutics Inc.; Waltham, MA. Available at: https://www.eloctate.com/ . Accessed July 19, 2019.

Elsevier’s Clinical Pharmacology Compendium. 2019. [Clinical Key Web site]. Available at: https://www.clinicalkey.com/pharmacology/ [via subscription only]. Accessed July 19, 2019.

FEIBA (Anti-Inhibitor Coagulant Complex). Baxter Healthcare Corporation. Westlake Village, CA. Prescribing information. 12/2018. Available at: www.feiba.com. Accessed July 19, 2019.

Glanzmann’s thrombasthenia. NORD: National Organization for Rare Disorders. Published 2018. Available at: http://rarediseases.org/rare-diseases/glanzmann-thrombasthenia/. Accessed July 22, 2019.

Grethlein SJ. Acquired Hemophilia Treatment & Management. [eMedicine Web site]. Treatment. 03/24/16. Available at: http://emedicine.medscape.com/article/211186-treatment#showall. Accessed July 19, 2019.

Helixate® FS. CSL Behring LLC Kankakee, IL. Prescribing information. 05/2016. Available at: http://www.helixatefs.com/ . Accessed July 19, 2019.

Hemlibra®. emicizumab-kxwh. Prescribing information. 10/2018. Genentech, Inc.; San Francisco, CA. Available at: https://www.hemlibra.com/ . Accessed July 19, 2019.

Hemophil M (Antihemophilic Factor [Human] Factor VIII). Lexington, MA: Baxalta US Inc. Prescribing information. 06/2018. Available at: https://www.shirecontent.com/PI/PDFs/HEMOFILM_USA_ENG.pdf . Accessed July 19, 2019.

Hoffman M. Recombinant factor VIIa: Clinical uses, dosing, and adverse effects. [UpToDate Web site]. 03/19/19. Available at:
http://www.uptodate.com/contents/therapeutic-uses-of-recombinant-coagulation-factor-viia [via subscription only]. Accessed July 22, 2019.

Hoots WK, Shapiro AD. Factor VIII and factor IX inhibitors in patients with hemophilia. 02/20/19. [UpToDate Web Site]. Available at [via subscription only]: http://www.uptodate.com/contents/factor-viii-and-factor-ix-inhibitors-in-patients-with-hemophilia. Accessed July 22, 2019.

Hoots WK, Shapiro AD. Treatment of bleeding and perioperative management in hemophilia A and B. 11/26/18. [UpToDate Web Site]. Available at [via subscription only]: http://www.uptodate.com/contents/treatment-of-hemophilia?source=see_link&sectionName=Factor+VIII+products&anchor=H11#H11. Accessed July 22, 2019.

Humate-P®. Antihemophilic factor/von Willebrand factor complex [human]. [package insert]. CSL Behring LLC Kankakee, IL.; 09/2017. Available at: http://www.humate-p.com/. Accessed July 22, 2019.

Idelvion®. Coagulation factor IX (recombinant). Prescribing information. 05/2018. CSL Behring LLC Kankakee, IL. Available at: http://www.idelvion.com/ . Accessed July 22, 2019.

Ixinity. Coagulation Factor IX (Recombinant). Prescribing information. 12/2018. Aptevo BioTherapeutics LLC: Berwyn, PA. Available at: https://www.ixinity.com/ . Accessed July 22, 2019.

Jivi®. Antihemophilic factor (recombinant) PEGylated-aucl. Prescribing information. 08/2018. Bayer Healthcare LLC: Whippany, NJ. Available at: http://labeling.bayerhealthcare.com/html/products/pi/Jivi_PI.pdf . Accessed July 19, 2019.

Koate® DVI. Antihemophilic Factor (Human), Factor VIII. Prescribing information. 08/2012. Grifols Therapeutics Inc. Research Triangle Park, NC. Available at: http://www.koate-dvi.com/en/web/koate-dvi . Accessed July 22, 2019.

Kogenate FS®. Antihemophilic factor (recombinant). Prescribing information. 05/2016. Available at: https://www.kogenatefs.com/home/prescribing-information. Available at: Accessed July 19, 2019.

Lexi-Drugs Compendium. 2019. [Lexicomp Online Web site]. Available at: http://online.lexi.com/lco/action/home [via subscription only]. Accessed July 22, 2019.

Lusher JM. Hemophilia: from plasma to recombinant factors. [American Society of Hematology Web site]. 12/2008. Available at: http://www.hematology.org/Publications/50-Years-in-Hematology/4737.aspx. Accessed July 22, 2019.

Ma AD, Carrizosa D. Acquired factor VIII inhibitors: pathophysiology and treatment. Hematology Am Soc Hematol Educ Program. 2006:432-7.

Mannucci PM. Rare inherited coagulation disorders. 09/18/18. [UpToDate Web site]. Available at: http://www.uptodate.com/contents/rare-recessively-inherited-coagulation-disorders?source=search_result&search=Rare+%28recessively+inherited%29+coagulation+disorders&selectedTitle=2%7E150 [via subscription only]. Accessed July 22, 2019.

Monoclate-P. Antihemophilic Factor (Human), Factor VIII:C. Prescribing information. 02/2014. CSL Behring LLC Kankakee, IL. Available at: http://www.cslbehring-us.com/products/Monoclate-P.htm . Accessed July 19, 2019.

Mononine®. Coagulation Factor IX (Human). Prescribing information. 12/2018. CSL Behring LLC Kankakee, IL. Available at: http://www.cslbehring-us.com/products/Mononine.htm . Accessed July 22, 2019.

NovoSevenRT®. Coagulation factor VIIa (recombinant). Prescribing information. 01/2019. Novo Nordisk Inc. Plainsboro, NJ. Available at: http://www.novosevenrt.com/. Accessed July 19, 2019.

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]. 04/23/18. Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=693. Accessed July 22, 2019.

National Hemophilia Foundation (NHF). Types of bleeding disorders. [NHF Web site]. Available at: https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders. Accessed July 22, 2019.

Novitas Solutions, Inc. Local Coverage Article: Billing and Coding: Hemophilia Factor Products (A56433) [Novitas Solutions, Inc. Medicare Services Web site]. Original: 07/01/2019. Available at: https://www.cms.gov/medicare-coverage-database/details/article-details.aspx?articleId=56433&ver=36&name=331*1&UpdatePeriod=843&bc=AAAAEAAAAAAA& . Accessed July 29, 2019.

Novitas Solutions, Inc. Local Coverage determination (LCD). LCD L35111: Hemophilia Factor Products. [Novitas Solutions, Inc. Medicare Services Web site]. Original: 10/01/2015. Revised: 04/01/2019. Available at:
https://www.cms.gov/medicare-coverage-database/details/lcd-details.aspx?LCDId=35111&ver=69&name=331*1&UpdatePeriod=830&bc=AAAAEAAAAAAA& . Accessed July 19, 2019.

Novoeight®. Coagulation factor VIII (recombinant). Prescribing information. 11/2018. Novo Nordisk Inc: Plainsboro, NJ. Available at: https://www.novoeight.com/ . Accessed July 19, 2019.

Nuwiq®. Coagulation factor VIII (recombinant). Prescribing information. 07/2017. Octapharma USA Inc: Hoboken, NJ. Available at: http://www.nuwiqusa.com/ . Accessed July 19, 2019.

Obizur. Antihemophilic Factor (Recombinant), Porcine sequence. Prescribing information. 09/2017. Lexington, MA: Baxalta US Inc. Available at: http://www.obizur.com/ . Accessed July 19, 2019.

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

Profilnine® SD. Coagulation factor IX complex (human). Prescribing information. 06/2018. Grifols Biologicals Inc: Los Angeles, CA. Available at: https://www.grifols.com/en/product/-/product/united-states/profilnine . Accessed July 22, 2019.

Rebinyn®. Coagulation factor IX (recombinant) GlycoPEGylated). Prescribing information. 05/2017. Novo Nordisk Inc. Plainsboro, NJ. Available at: https://www.rebinyn.com/ . Accessed July 22, 2019.

RECOMBINATE®. Antihemophilic factor, recombinant. Prescribing information. 06/2018. Lexington, MA: Baxalta US Inc. Available at: http://www.recombinate.com/ . Accessed July 19, 2019.

RiaSTAP®. [Package Insert]. Labeling revised 04/2019. Kankakee, IL: CSL Behring LLC. Available at: http://www.riastap.com/. Accessed July 19, 2019.

Rixubis®. Coagulation Factor IX (Recombinant). Prescribing information. 05/2018. Baxter US Inc: Lexington, MA. Available at: https://www.rixubis.com/ . Accessed July 22, 2019.

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: 1639-1657.

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

Schwartz RA. Factor IX Deficiency (Hemophilia B) Medication. [eMedicine Web site]. 04/02/18. Available at: http://emedicine.medscape.com/article/199088-medication#showall. Accessed July 22, 2019.

Schwartz RA. Factor X. [eMedicine Web site]. 03/23/18. Available at: http://emedicine.medscape.com/article/209867-overview. Accessed July 22, 2019.

Schwartz RA. Factor XIII Deficiency Treatment & Management. [eMedicine Web site]. 04/02/18. Available at: http://emedicine.medscape.com/article/209179-treatment. Accessed July 22, 2019.

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 Feb;138(2):278-81.

Tretten®. Coagulation Factor XIII A-Subunit (Recombinant). Prescribing information. 11/2016. Novo Nordisk Inc. Plainsboro, NJ. Available at: http://www.tretten.com/ . Accessed July 22, 2019.

Truven Health Analytics Inc. Micromedex® 2.0. DrugDex. Antihemophilic Factor (various). [Micromedex Web site]. 2019. Available at: http://www.micromedexsolutions.com/micromedex2/librarian/ [via subscription only]. Accessed July 22, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Coagulation Factor X (Human), Coagadex®. Product information and other documents. [FDA Web site]. 09/2018. Available at: http://www.fda.gov/biologicsbloodvaccines/bloodbloodproducts/approvedproducts/licensedproductsblas/fractionatedplasmaproducts/ucm468110.htm. Accessed July 22, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Antihemophilic factor (recombinant), plasma/albumin free (XYNTHA®). Product information and other documents. [FDA Web site]. Approved 10/17/2014. Available at: https://www.fda.gov/vaccines-blood-biologics/approved-blood-products/xyntha . Accessed July 22, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Antihemophilic factor, recombinant (Refacto®). Prescribing information & supplemental biologics license approval letter. [FDA Web site]. Available at: http://www.fda.gov/biologicsbloodvaccines/bloodbloodproducts/approvedproducts/licensedproductsblas/fractionatedplasmaproducts/ucm089061.htm . Accessed July 19, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Antihemophilic factor, recombinant, glycopegylated-exei (Esperoct). Prescribing information & supplemental biologics license approval letter. [FDA Web site]. Approved 02/19/2019. Available at: https://www.fda.gov/vaccines-blood-biologics/esperoct . Accessed July 19, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Coagulation factor VIII (recombinant) (Kovaltry®). Product information and other documents. [FDA Web site]. Approved 03/16/16. Available at: http://www.fda.gov/biologicsbloodvaccines/bloodbloodproducts/approvedproducts/licensedproductsblas/fractionatedplasmaproducts/ucm491116.htm. Accessed July 19, 2019.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Coagulation factor VIIa (recombinant) (NovoSeven®). Package insert & supplemental biologics license approval letter. [FDA Web site]. Princeton, NJ: Novo Nordisk, Inc. Available at: http://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm089228.htm (label updated 10/2006)

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Fibrinogen (Human). Fibryna®). Prescribing information. [FDA Web site]. Product Approved 06/07/2017. Available at: https://www.fda.gov/biologicsbloodvaccines/bloodbloodproducts/approvedproducts/licensedproductsblas/fractionatedplasmaproducts/ucm562342.htm . Accessed July 19, 2019.

Vonvendi. von Willebrand Factor (Recombinant). Prescribing information. 02/2019. Baxalta US Inc: Lexington, MA.
Available at: http://www.vonvendi.com/ . Accessed July 22, 2019.

Wilate®. von Willebrand factor/coagulation factor VIII complex [human]). Prescribing information. 09/2016. Octapharma USA, Inc.: Hoboken, NJ. Available at: https://www.wilateusa.com/ . Accessed July 22, 2019.

Wilczynski C. Fibrinogen. [eMedicine Web site]. 02/12/14. Available at: http://emedicine.medscape.com/article/2085501-overview#showall . Accessed July 22, 2019.

Zaiden RA. Hemophilia B. 06/08/17. Available at: http://emedicine.medscape.com/article/779434-overview. Accessed July 22, 2019.




Coding

Inclusion of a code in this table does not imply reimbursement. Eligibility, benefits, limitations, exclusions, precertification/referral requirements, provider contracts, and Company policies apply.

The codes listed below are updated on a regular basis, in accordance with nationally accepted coding guidelines. Therefore, this policy applies to any and all future applicable coding changes, revisions, or updates.

In order to ensure optimal reimbursement, all health care services, devices, and pharmaceuticals should be reported using the billing codes and modifiers that most accurately represent the services rendered, unless otherwise directed by the Company.

The Coding Table lists any CPT, ICD-9, ICD-10, and HCPCS billing codes related only to the specific policy in which they appear.

CPT Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD - 10 Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD -10 Diagnosis Code Number(s)

D66 Hereditary factor VIII deficiency

D67 Hereditary factor IX deficiency

D68.0 Von Willebrand's disease

D68.2 Hereditary deficiency of other clotting factors

D68.311 Acquired hemophilia

D68.312 Antiphospholipid antibody with hemorrhagic disorder

D68.318 Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors

D68.4 Acquired coagulation factor deficiency

D69.1 Qualitative platelet defects



HCPCS Level II Code Number(s)



MEDICALLY NECESSARY

J7170 Injection, emicizumab-kxwh, 0.5 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

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

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.


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 NovoSeven®, NovoSevenRT®, Eptacog Beta, activated:

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


THE FOLLOWING CODE IS USED TO REPRESENT Koate®DVI, Hemophil M, Monoclate-P®:

J7190 Factor VIII (antihemophilic factor, human) per IU


THE FOLLOWING CODE IS USED TO REPRESENT Advate®, Afstyla®, Esperoct, Helixate® FS, Kogenate FS®, Recombinate®, ReFacto®:

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


THE FOLLOWING CODE IS USED TO REPRESENT Alphanine® SD, Mononine®:

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


THE FOLLOWING CODE IS USED TO REPRESENT Profilnine® SD:

J7194 Factor IX complex, per IU


THE FOLLOWING CODE IS USED TO REPRESENT BeneFIX®, Ixinity®:

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


NOT ELIGIBLE FOR REIMBURSEMENT

THE FOLLOWING CODE REPRESENTS HYATE:C® WHICH IS NO LONGER MANUFACTURED AND HAS BEEN WITHDRAWN FROM THE MARKET:

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


THE FOLLOWING CODE REPRESENTS BEBULIN WHICH IS NO LONGER MANUFACTURED AND HAS BEEN WITHDRAWN FROM THE MARKET:

J7194 Factor IX complex, per IU




Revenue Code Number(s)

N/A

Coding and Billing Requirements


Cross References

Related Documents


Policy History

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 (ra®) 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.
Version Effective Date: 10/21/2019
Version Issued Date: 10/21/2019
Version Reissued Date: N/A

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