360 Form

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Pharmacy Medical Policy Factor and Non-Factor Anti-Hemophilic Drugs Table of Contents
• Policy: Commercial • Policy History • Endnotes
• Policy: Medicare • Information Pertaining to All Policies
• Forms • Coding Information
• References

Policy Number: 360

Related Policies None

Prior Authorization Information ☒ Prior Authorization ☐ Step Therapy ☐ Quality Care Dosing

Pharmacy Operations: Tel: 1-800-366-7778 Fax: 1-800-583-6289 Policy last updated 1/15/2026 Pharmacy (Rx) or Medical (MED) benefit coverage ☐ Rx ☒ MED To request for coverage: Physicians may call, fax, or mail the attached form (Formulary Exception/Prior Authorization form) to the address below.
Blue Cross Blue Shield of Massachusetts Pharmacy Operations Department 25 Technology Place Hingham, MA 02043

Individual Consideration: Policy for requests that do not meet clinical criteria of this policy, see section labeled Individual Consideration
Policy applies to Commercial Members:
• Managed Care (HMO/POS) • PPO/EPO
• Indemnity
• MEDEX with Rx plans • Managed Blue for Seniors Policy does NOT apply to: • Medicare Advantage

We may cover:

Anti-Inhibitor • Anti-Inhibitor Coagulant Complex is indicated for the control of spontaneous bleeding episodes or to cover surgical interventions in hemophilia A and hemophilia B patients.

Antithrombin-Directed Double-Stranded Small Interfering Ribonucleic Acid (siRNA) Agent

2 • Routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adult and pediatric patients aged 12 years and older with hemophilia A or B with or without factor VIII or IX inhibitors Factor VII • Coagulation factor indicated for the treatment of bleeding episodes and perioperative management in adults and children with hemophilia A or B with inhibitors, congenital Factor VII (FVII) deficiency, and Glanzmann’s thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets & Treatment of bleeding episodes and perioperative management in adults with acquired hemophilia.

Factor VIII • Human anti-hemophilic factor (AHF) maintenance therapy (prophylaxis) as needed to maintain trough levels at 1% or greater in patients with severe Hemophilia A (AHF activity less than 1% of normal).1 • Human anti-hemophilic factor (AHF) for treatment and/or management of bleeding episodes in surgical patients with mild hemophilia (AHF activity 5%-30%) or moderately severe hemophilia (AHF activity 1%-5%) 2. • Human anti-hemophilic factor (AHF) for treatment of moderate to severe Von Willebrand’s disease in appropriate settings, for example, major surgery, trauma.2 • Human anti-hemophilic factor (AHF) for acquired factor VIII deficiency.3 • On-demand treatment and control of bleeding episodes

Factor IX • Coagulation Factor IX concentrate indicated in adults and children with hemophilia B (congenital Factor IX deficiency) control and prevention of bleeding episodes, perioperative management, and routine prophylaxis control and prevention of bleeding episodes. • Coagulation Factor IX [Recombinant] is an antihemophilic factor indicated in adults and children with hemophilia B for: Control and prevention of bleeding episodes, perioperative management, on-demand treatment, and routine prophylaxis. • Blood coagulation factor replacement indicated for the urgent reversal of acquired coagulation factor deficiency induced by Vitamin K antagonist (VKA, e.g., warfarin) therapy in adult patients.

Factor X • Plasma-derived human blood coagulation Factor X indicated in adults and children (aged 12 years and above) with hereditary Factor X deficiency for On-demand treatment and control of bleeding episodes • Plasma-derived human blood coagulation Factor X indicated in adults and children (aged 12 years and above) with hereditary Factor X deficiency for Perioperative management of bleeding in patients with mild or moderate hereditary Factor X deficiency.

Factor XIII • Routine prophylaxis for bleeding in patients with congenital factor XIII A-subunit deficiency.

Factor IXa- and factor X-directed antibody • Factor IXa- and factor X-directed antibodies are indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adult and pediatric patients with hemophilia A (congenital factor VIII deficiency) with or without factor VIII inhibitors.

Fibrogen Concentrate • Fibrinogen Concentrate indicated for the treatment of acute bleeding episodes in patients with congenital fibrinogen deficiency.
Tissue factor pathway inhibitor (TFPI) antagonist: Alhemo • Routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adult and pediatric patients 12 years of age and older with: hemophilia A (congenital factor VIII deficiency)

3 with or without FVIII inhibitors OR hemophilia B (congenital factor IX deficiency) with or without FIX inhibitors. Tissue factor pathway inhibitor (TFPI) antagonist: Hympavzi • Routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adult and pediatric patients 12 years of age and older with: hemophilia A (congenital factor VIII deficiency) without FVIII inhibitors OR hemophilia B (congenital factor IX deficiency) withOUT FIX inhibitors. Recombinant Von Willebrand Factor • Treatment (on demand) and control of bleeding episodes in adult and pediatric patients with von Willebrand disease • Perioperative management of bleeding in adult and pediatric patients with von Willebrand disease • Routine prophylaxis in adult patients to reduce the frequency of bleeding episodes in adults with von Willebrand disease

We do not cover Factor and Non-Factor Anti-Hemophilic Drugs for conditions other than those listed above.

CPT Codes / HCPCS Codes / ICD Codes Inclusion or exclusion of a code does not constitute or imply member coverage or provider reimbursement. Please refer to the member’s contract benefits in effect at the time of service to determine coverage or non-coverage as it applies to an individual member.

Providers should report all services using the most up-to-date industry-standard procedure, revenue, and diagnosis codes, including modifiers where applicable.

The following codes are included below for informational purposes only; this is not an all-inclusive list.

The above medical necessity criteria MUST be met for the following codes to be covered for Commercial Members: Managed Care (HMO and POS), PPO, and Indemnity:

CPT Codes There is no specific CPT code for this service.

HCPCS Codes HCPCS codes: Code Description J7174 Injection, fitusiran, 0.04 mg (Qfitlia) C9132 Factor IX : Prothrombin complex concentrate (human), per i.u. of Factor IX activity (Kcentra) J7173 Injection, concizumab-mtci, (Alhemo) C9304
Injection, marstacimab-hncq, 0.5 mg (Hympavzi) C9399 Unclassified drugs or biologicals (NOC) (i.e. Hemlibra) J7170 Injection, emicizumab-kxwh, 0.5 mg J7175 Injection, factor x, (human), 1 i.u.(Coagadex) J7177 Injection, human fibrinogen concentrate (fibryga), 1 mg J7178 (RiaSTAP) J7179 Injection, Von Willebrand factor (recombinant), 1 i.u. vwf:rco (Vonvendi) J7178 Injection, human fibrinogen concentrate, not otherwise specified, 1 mg J7180 Injection, factor XIII (antihemophilic factor, human), 1 IU (Corifact) J7181 Injection, factor XIII A-subunit, (recombinant), per IU (Tretten) J7182 Injection, factor VIII, (antihemophilic factor, recombinant), (NovoEight), per IU J7183 Injection, von Willebrand factor complex (human), 1 IU VWF (Wilate)

4 J7185 Injection, factor VIII (antihemophilic factor, recombinant) (XYNTHA), per IU J7186 Injection, antihemophilic factor VIII/von Willebrand factor complex (human), per factor VIII i.u. J7187 Injection, von Willebrand factor complex (Humate-P), per IU VWF:RCO J7188 Injection, factor viii (antihemophilic factor, recombinant), (OBIZUR), per i.u. J7189 Factor VIIa (antihemophilic factor, recombinant), per 1 microgram (Novoseven RT, Sevenfact, Alphanate) J7190 Factor VIII (antihemophilic factor [human]) per IU (Hemofil, Koate, Monoclate) J7191 Factor VIII (antihemophilic factor [porcine]) per IU J7192 Factor VIII (antihemophilic factor, recombinant) per IU, not otherwise specified (Advate, Helixate FS, Kogenate FS, Kovaltry, Recombinate) J7193 Factor IX (antihemophilic factor, purified, non-recombinant) per IU (AlphaNine, Mononine) J7194 Factor IX, complex, per IU (Profilnine) J7195 Factor IX (antihemophilic factor, recombinant) per IU (BeneFIX, Ixinity) (NOC) J7198 Anti-inhibitor, per IU Injection, antiinhibitor Coagulant Complex (NOC) (Feiba) J7199 Hemophilia clotting factor, not otherwise classified (NOC) (Rebinyn, Esperoct, or Altuviiio) J7200 Injection, factor IX, (antihemophilic factor, recombinant), Rixubis, per IU J7201 Injection, factor IX, FC fusion protein (recombinant) (Alprolix), per IU
J7202 Injection, factor ix, albumin fusion protein, (recombinant), 1 i.u. (Idelvion) J7203 Injection factor ix, (antihemophilic factor, recombinant), glycopegylated, (Rebinyn), 1 iu J7205 Injection, factor viii fc fusion (recombinant), per iu (Eloctate) J7207 Injection, factor viii, (antihemophilic factor, recombinant), pegylated, 1 i.u. (Adynovate) J7208 Injection, factor viii, (antihemophilic factor, recombinant), pegylated-aucl, (jivi), 1 i.u. J7209 Injection, factor viii, (antihemophilic factor, recombinant), 1 i.u. (Nuwiq) 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 J3590 Unclassified biologics (NOC) (i.e. Hemlibra, Hympavzi)

Other Information Preferred Home Infusion Therapy Network Referring providers are encouraged to use these preferred Home Infusion providers to obtain these medications.

Preferred Home Infusion Therapy Provider Contact Information:

AccredoSM
Phone: 1-877-988-0058 Website: www.accredo.com

Boston Hemophilia Center
(only for members who receive medical care with their affiliated clinics at Boston Children's Hospital and Brigham and Women's Hospital)

Phone: 1-617-355-6101 (Boston Children's Hospital/pediatric) Website: Boston Hemophilia Center

Phone: 1-617-278-0707 (Brigham & Women’s Hospital/adult) Website: Boston Hemophilia Center

5 Caremark, LLC. Phone: 1-866-846-3096 Website: www.caremark.com

Coram™ Specialty Infusion Services Phone: 1-800-678-3442 (for hemophilia therapies only: 1-888-699-7440) Website: www.coramhc.com

Mass General Hemophilia and Thrombosis Treatment Center Phone: 1-877-726-5130 (Adult Hemophilia Program) Website: www.massgeneral.org

Phone: 1-617-726-2737 (Pediatric Hemophilia Program) Website: www.massgeneral.org

Individual Consideration All our medical policies are written for the majority of people with a given condition. Each policy is based on medical science. For many of our medical policies, each individual’s unique clinical circumstances may be considered in light of current scientific literature. Physicians may send relevant clinical information for individual patients for consideration to:

Blue Cross Blue Shield of Massachusetts Pharmacy Operations Department 25 Technology Place Hingham, MA 02043
Tel: 1-800-366-7778 Fax: 1-800-583-6289

Policy History Date Action 1/15/2026 Updated Vonvendi’s expanded indication. 11/2025 Updated FDA labeling for Alhemo and updated formatting and references. 10/2025 Updated HCPCS codes for Qfitlia and Alhemo 7/2025 Added Qfitlia to the policy. Removed Bebulin as it was discontinued. Updated references. 5/2025 Added Alhemo and Hympavzi to the policy with references, updated policy name and HCPCS codes. 3/2024 Included links to Standard PA form and HIT form. 7/2023 Reformatted Policy. 1/2021 Coding information clarified. 10/2020 Removed deleted codes 2/2020
Added Esperoct® to the policy
7/2019 Clarified coding information. 1/2019 Clarified coding changes. 11/2018 Updated to include a new indication for Factor X deficiency and new indication for Factor IXa- and factor X-directed antibody class. 7/2018 Clarified coding information. 5/2018 Updated to include Hemlibra. 1/2018 Clarified coding information. 6/2017 Updated address for Pharmacy Operations. 1/2017 Updated to add New HCPCS/CPT codes. 10/2016 Updated to include new HCPCS/CPT codes. 4/2016 Updated to include Factor X criteria & new CPT codes.

6 1/2016 Updated to include Obizur & Eloctate approved HCPCS codes. 6/2015 New Format instituted. Also updated to include Factor VII, Factor IX, Factor XIII, Fibrogen Concentrate, and Anti-Inhibitor to the policy plus add ICD10. 1/2013 Updated 1/2013 to include new FDA approved products AlphaNine® SD, Bebulin® VH, BeneFix®, Corifact® and Wilate®. Updated to add new HCPCS code C9133. 3/2011 Updated 3/11 to include preferred HIT provider contact information.
9/2010 Reviewed 9/2010 MPG-Hematology and Oncology, no changes in coverage were made.
1/2010 Updated, 1/1/2010 to add revised language to J7192 code. 10/2009 Updated 10/09 to reflect UM requirements. 9/2009 Updated 9/09 based on review of BCBSA #2.01.13, no change in policy statement. 10/2008 Reviewed 10/08 MPG – Hematology/Oncology, no changes in coverage were made.
9/2007 Reviewed 9/07 MPG hematology/oncology, no changes in coverage were made. 9/2006 Reviewed 9/06 MPG-Hematology/Oncology, no changes in coverage were made. 2/2006 Updated 2/06 to include preferred home infusion vendor for hemophilia. 9/2005 Reviewed 9/05 MPG Hematology/Oncology, no changes in coverage were made. 9/2003 Reviewed 9/03 MPG hematology/oncology, no changes in coverage were made.
9/2002 Reviewed 9/02 MPG hematology/oncology, no changes in coverage were made. 9/2001 Reviewed 9/01, no changes in coverage were made. 8/2000 Updated 8/00 to include coverage for acquired factor VIII deficiency, effective 2/01.
8/1999 Reviewed 8/99 to include coverage for human anti-hemophilic factor (AHF) for treatment and/or management of bleeding episodes in surgical patients with mild hemophilia (AHF activity 5%-30%) or moderately severe hemophilia (AHF activity 1%-5%) and for treatment of moderate to severe Von Willebrand’s disease in appropriate settings, for example, major surgery, trauma. 8/1998 Updated 8/98 to clarify that Human Anti-hemophilic Factor is not covered for conditions other than Factor VIII deficiency. No changes in coverage were made. . 2/1997 Reviewed 2/97 following a literature search for 1996; additional scientific background information was added. 9/1995 Issued 9/95.

References

1. Advate® [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; March 2025.
2. Alhemo® [package insert] Plainsboro, NJ: Novo Nordisk, Inc.; 7/2025.
3. Alphanate® [package insert]. Los Angeles, CA: Grifols Biologicals Inc.; November 2022.
4. Alphanate® SD [package insert]. Los Angeles, CA: Grifols Biologicals Inc.; November 2022.
5. Profilnine® [package insert]. Los Angeles, CA: Grifols Biologicals Inc.; November 2022.
6. Rixubis® [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; Sept. 2014.
7. Alphanine® SD [package insert] . Los Angeles, CA: Grifols Biologicals Inc.; Jan 2004.
8. Alprolix™ [package insert] ]. Cambridge, MA: Biogen Idec, Inc.; October 2020.
9. Benefix® [package insert] Philadelphia, PA: Wyeth Pharmaceuticals Inc.; April 2021.
10. Corifact® [package insert] Kankakee, IL: CSL Behring LLC.; September 2020.
11. Eloctate™ [package insert] . Cambridge, MA: Biogen Idec, Inc.; May 2023.
12. Feiba® [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; Nov 2013.
13. Feiba® NF [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; Feb 2011.
14. Feiba® VH [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; Apr 2005
15. Helixate® FS [package insert] Kankakee, IL: CSL Behring LLC.; May 2016.
16. Hemofil® M [package insert] Cambridge, MA: Takeda Pharmaceuticals; February 2025.
17. Humate-P® [package insert] Kankakee, IL: CSL Behring LLC.; June 2020.
18. Hympavzi® [package insert] New York, NY: Pfizer, Inc.; October 2024.
19. Kcentra® [package insert] Kankakee, IL: CSL Behring LLC.; May 2023.
20. Koate-DVI® [package insert]. Los Angeles, CA: Grifols Biologicals Inc.; Aug 2012.
21. Kogenate™ FS [package insert]. Tarrytown, NY: Bayer Healthcare.; May 2014.
22. Monoclate-P® [package insert] Kankakee, IL: ZLB Behring LLC.; February 2014.

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23. Mononine® [package insert] Kankakee, IL: CSL Behring LLC.; December 2018.
24. Novoseven® RT [package insert]. Bagsvaerd, Denmark: Novo Nordisk A/S.; July 2020.
25. Obizur® [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; February 2023.
26. Qfitlia® [package insert] Cambridge, MA: Genzyme Corporation.; March 2025
27. Recombinate® [package insert] Westlake Village, CA: Baxter Healthcare Corporation.; Dec 2010.
28. RiaSTAP® [package insert] Kankakee, IL: CSL Behring LLC.; June 2021.
29. Tretten® [package insert]. Bagsvaerd, Denmark: Novo Nordisk A/S.; June 2020.
30. Wilate® [package insert] Hoboken, NJ: Octapharma USA Inc.; December 2023.
31. Xyntha® [package insert] Philadelphia, PA: Wyeth Pharmaceuticals Inc.; August 2020.
32. Idelvion® [package insert] Kankakee, IL: CSL Behring LLC.; June 2023.
33. NuwiQ® [package insert] Hoboken, NJ: Octapharma USA Inc.; December 2024.
34. VonVendi® [package insert] Westlake Village, CA: Baxalta US Inc.; September 2025.

35. Hemlibra® [package insert] South San Francisco, CA: Genentech, Inc.; Nov 2017.

    Endnotes

36. Based on the TEC (Technology Evaluation Center) 6/95 assessment of medical literature from 1970- 1995, including the National Hemophilia Foundation recommendations from 1994. Reports by Aledort (1994, n=477), Manco-Johnson (1994, n=13), Nilsson (1992 n=87), Petrini (1991, n=14), Peterson (1981 n=59), and Schramm (1993, n=87) were reviewed. Joint damage and quality of life were addressed by most studies. Aronstam (1976, 1977, 1979) reported comparisons of multiple dosing schedules for maintenance therapy. Maintenance therapy was shown to improve frequency of bleeding, orthopedic outcomes, hospitalizations, and school/work attendance primarily through reduction in joint bleeding. Success is best documented for primary prevention, but secondary prevention does appear to result in reduced further damage to joints.
37. A cost-effectiveness analysis entitled, Episodic versus prophylactic infusions for hemophilia A: a cost-effectiveness analysis was reported by Smith PS et al. in J Pediatrics 1996 Sep;129(3):424-31.
    This multi-center analysis collected charge data from 70 patients treated at 11 US hemophilia treatment centers. A model was constructed for hypothetical patients aged 3 to 50, and three different infusion models were considered. The prophylactic group would receive more drug, but sustain fewer bleeding episodes. The total cost of prophylactic care from ages 3 through 50 would equal the cost of episodic care only if the price of the concentrate were reduced by 50%. While prophylactic care markedly reduces bleeding events and should preserve joint function, a substantial cost is incurred..
38. Recommendations through Electric Blue Review from Peter Marks, MD Brigham and Women’s Hospital, Boston, Massachusetts; Douglas Taylor, MD, Lawrence Memorial Hospital.
39. Recommendations through Electric Blue Review from Margot Kruskall, MD, Beth Israel Hospital.

40. Guidelines for the Management of Hemophilia -2nd Edition - Treatment Guidelines Working Group World Federation of Hemophilia (WFH) Use this link for Outpatient Buy & Bill:

    To request prior authorization using the Massachusetts Standard Form for Medication Prior Authorization Requests (eForm), click the link below: http://www.bluecrossma.org/medical-policies/sites/g/files/csphws2091/files/acquiadam- assets/023%20E%20Form%20medication%20prior%20auth%20instruction%20prn.pdf Use this link for Home infusion: Home Infusion Therapy Prior Authorization Form 430