CurrentEmblemHealthPolicy MG.MM.PH.413

Beqvez (fidanacogene elaparvovec-dzkt kit) Intravenous Infusion

Clinical coverage policy for one-time, per-lifetime administration of Beqvez (gene therapy) for adults with moderate to severe hemophilia B, specifying eligibility criteria, documentation, dosing, limits, applicable codes, and lab/monitoring requirements.

Policy Summary

PayerEmblemHealth

PolicyBeqvez (fidanacogene elaparvovec-dzkt kit) Intravenous Infusion

Policy CodePolicy MG.MM.PH.413

Change TypeAnnual Review; New Policy (origin 06/28/2024)

Effective DateJun 28, 2024

Next Review Date

Key ActionPrescribing physician must provide documentation to demonstrate all eligibility criteria (labs, inhibitor testing, history of therapy/exposure days, absence of neutralizing antibodies, body weight, specialist prescription) for preauthorization.

SourceLink

POLICY UPDATE CHANGES

Annual Review no updates noted in revision history (2/27/2025).

Policy created/originated 06/28/2024.

1Dose per lifetime

>=18Minimum patient age (years)

<2%Factor IX level threshold for eligibility

150Required Factor IX exposure days

This clinical coverage policy addresses one-time, per-lifetime administration of Beqvez (fidanacogene elaparvovec-dzkt), an AAV vector–based gene therapy, for adults with moderate to severe hemophilia B who have a history of life‑threatening hemorrhage, use Factor IX prophylaxis, or experience repeated serious spontaneous bleeding. Coverage is provided as a single intravenous infusion at a dose of 5 x 10^11 vector genomes per kg (one dose per lifetime) and the policy stance is covered with criteria requiring documentation of eligibility, labs, testing, and specialist prescribing to approve treatment.

Key thresholds / facts

Dose per lifetime1 (one-time, per-lifetime single dose)

Dose and routeSingle IV infusion of 5 x 10^11 vector genomes/kg

Minimum patient age>= 18 years

Factor IX activity level thresholdBaseline Factor IX level < 2% of normal

Required Factor IX exposure days> 150 exposure days (history of Factor IX use)

Initial Therapy Criteria (Hemophilia B)

Initial Criteria (Hemophilia B)

Approve a one-time (per lifetime) single dose if the patient meets ALL of the following:

ALL of the following

Demographics and prior therapy
- Patient is male
- Patient is > 18 years of age
- Prior gene therapy receipt: Patient has not received a gene therapy for hemophilia B
  Verification in claims history required; if claims history unavailable, prescribing physician must confirm no prior receipt of Beqvez or Hemgenix
- Baseline factor IX level: Patient has moderately severe or severe hemophilia B as evidenced by a baseline (without Factor IX replacement therapy) Factor IX level < 2% of normal< 2% of normal
  Documentation required
- History of bleeding or prophylaxis
  - Patient has current or historical life-threatening hemorrhage
  - Patient currently uses factor IX prophylaxis therapy
  - Patient has repeated, serious spontaneous bleeding episodes
- Factor IX exposure days: According to the prescribing physician, the patient has a history of use of Factor IX therapy for > 150 exposure days> 150 exposure days
  Per prescribing physician
AAV neutralizing antibody status: Patient does not have neutralizing antibodies to adeno-associated virus serotype Rh74var (AAVRh74var) capsid as detected by an FDA-approved test
Documentation required
Factor IX inhibitor testing and status
- Factor IX inhibitor titer testing has been performed within 30 dayswithin 30 days
  Documentation required
- Patient is negative for Factor IX inhibitors
  Documentation required
- Patient does not have a history of Factor IX inhibitors
  Documentation required
Post-treatment factor IX therapy plan: Prophylactic therapy with Factor IX will not be given after Beqvez administration once adequate Factor IX levels have been achieved
Use of episodic Factor IX therapy is acceptable for treatment of bleeds and for surgery/procedures as determined by hemophilia specialist physician
Infection/antiviral status
- Patient does not have an active infection with hepatitis B virus or hepatitis C virus
  Documentation required
- Patient is not currently receiving antiviral therapy for a prior hepatitis B virus or hepatitis C virus exposure
  Documentation required
Liver function tests (within 30 days)
- ALT: Alanine aminotransferase (ALT) level is < two times the upper limit of normal< 2x ULN
  Documentation required
- AST: Aspartate aminotransferase (AST) level is < two times the upper limit of normal< 2x ULN
  Documentation required
- Alkaline phosphatase level is < two times the upper limit of normal< 2x ULN
  Documentation required
- Total bilirubin level is < 1.5 times the upper limit of normal
Renal and hematologic tests (within 30 days)
- Creatinine: Within 30 days, creatinine was < 2.0 mg/dL< 2.0 mg/dL
  Documentation required
- Platelet count: Within 30 days, platelet count was > 100 x 10^9/L> 100 x 10^9/L
  Documentation required
Prescribing provider: Medication is prescribed by a hemophilia specialist physician
Body weight documentation: Current patient body weight has been obtained within 30 dayswithin 30 days
Documentation required; dose based on adjusted body weight for BMI > 30 kg/m^2 using: Dose Weight (kg) = 30 kg/m^2 x [Height (m)]^2
Approval and dosing: If criteria A through P are met, approve one dose (vials in a kit) of Beqvez to provide a one-time (per lifetime) single dose of 5 x 10^11 vector genomes per kg of body weight by intravenous infusion5 x 10^11 vector genomes/kg (single dose, one-time per lifetime)
Verification required; Table 1 provides number of vials per kit and NDCs
Medicare note: Note: Factor IX inhibitor and other documentation requirements do not apply for Medicare

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Provider Actions and Requirements

Prior Authorization

Preauthorization required with documentation

Prescribing physician must provide documentation to demonstrate all eligibility criteria (labs, inhibitor testing, history of therapy/exposure days, absence of neutralizing antibodies, body weight, specialist physician prescription). Verification in claims history required for prior receipt of Beqvez or Hemgenix; if claims history unavailable, physician attestation required.

Affected codes: J1414; applicable NDCs (kits/vials): 00069-0422-01, 00069-2004-XX, 00069-2005-XX, 00069-2006-XX, 00069-2007-XX
Verification requirements: check claims history for prior receipt of Beqvez or Hemgenix; if claims history not available, obtain prescribing physician attestation confirming no prior receipt

Documentation Required

Laboratory and testing documentation

Require documentation within 30 days for Factor IX inhibitor titer testing, ALT, AST, alkaline phosphatase, total bilirubin, platelet count, creatinine, and body weight; and documentation of AAVRh74var neutralizing antibody test (FDA-approved) and baseline Factor IX level.

Factor IX inhibitor titer testing (performed within 30 days)
Alanine aminotransferase (ALT) (within 30 days)
Aspartate aminotransferase (AST) (within 30 days)
Alkaline phosphatase (within 30 days)
Total bilirubin (within 30 days)
Platelet count (> 100 x 10^9/L within 30 days)
Creatinine (< 2.0 mg/dL within 30 days)
Current body weight (obtained within 30 days)
AAVRh74var neutralizing antibody test (FDA-approved) — patient must be negative
Baseline (without Factor IX replacement) Factor IX level

Billing Rule

One-time per lifetime dosing limit

Coverage provided for one dose per lifetime: single dose of 5 x 10^11 vector genomes per kg by intravenous infusion; verify number of vials per kit and bill per therapeutic dose using J1414 and appropriate NDCs.

One dose per lifetime (single intravenous infusion)
Dose amount: 5 x 10^11 vector genomes per kg (one-time)
Verify number of vials per kit (see Table 1 / NDCs) — confirm kit vial count and corresponding NDC
Bill per therapeutic dose using HCPCS J1414 and the appropriate NDC(s) for the kit/vials

Denial Risk

Denial risk for prior gene therapy or positive AAV neutralizing antibodies

Claims may be denied if member has prior receipt of gene therapy for hemophilia B or has neutralizing antibodies to AAVRh74var as detected by an FDA-approved test.

Denial risk for prior receipt of gene therapy for hemophilia B (verify claims history)
Denial risk if patient is positive for AAVRh74var neutralizing antibodies as detected by an FDA-approved test

Applicable Codes

Applicable HCPCS/J-codeHCPCSCovered

J1414

Injection, fidanacogene elaparvovec-dzkt, per therapeutic dose

Applicable NDCs (kits/vials)NDCCovered

00069-0422-01	Beqvez 1X10E13/ML
00069-2004-XX
00069-2005-XX	Beqvez 5x1 mL
00069-2006-XX	Beqvez 6x1 mL
00069-2007-XX	Beqvez 2x1 mL

Applicable ICD-10 DiagnosesICD-10Covered

D67	Hereditary Factor IX Deficiency

Clinical Evidence & References

Evidence and references:

Prescribing information: Beqvez intravenous infusion, Pfizer; April 2024.

FDA‑approved testing requirement: patients must be negative for neutralizing antibodies to AAVRh74var (AAVRh74var) capsid as detected by an FDA‑approved test (presence is exclusionary).

Applicable billing/coding references: HCPCS J1414 (Injection, fidanacogene elaparvovec‑dzkt, per therapeutic dose) and listed NDCs for Beqvez kits support billing per therapeutic dose.

Background and Definitions

Background: Beqvez is an adeno‑associated virus (AAV) vector–based gene therapy indicated for adults with moderate to severe hemophilia B (congenital Factor IX deficiency) who have life‑threatening hemorrhage history, use Factor IX prophylaxis, or have repeated serious spontaneous bleeding episodes. Treatment is restricted to patients who are negative for neutralizing antibodies to AAVRh74var as detected by an FDA‑approved test. The recommended regimen is a single intravenous infusion delivering a one‑time dose of 5 x 10^11 vector genomes per kg (dose weight adjustment specified for BMI > 30 kg/m2).

Definitions:

AAVRh74var neutralizing antibody: Neutralizing antibodies to adeno‑associated virus serotype Rh74var as detected by an FDA‑approved test; presence is exclusionary.

Revision History

2024-06-28policy_created

Policy created/originated 06/28/2024

2025-02-27annual_reviewLatest

Annual Review no updates noted in revision history (2/27/2025)