Medical Drug Criteria (MDC)

Policy Num:       P1.001.001
Policy Name:     Adakveo® (crizanlizumab-tmca)
Policy ID:          [P1.001.001] [Ac /L / M+/ P+ ][0.00.00]

Last Review:       September 22, 2025
Next Review:      September 20, 2026
 

Related Policies: 

None

Adakveo® (crizanlizumab-tmca)

Summary

Adakveo® is an IV P-selectin inhibitor approved by the FDA for reducing vaso-occlusive crises (VOC) in patients with sickle cell disease (SCD) aged ≥16 years

Objective

Ensure evidence-based, safe, and clinically appropriate use of Adakveo® in patients with SCD.

Policy Statements

Adakveo may be considered medically necessary in patients 16 years of age or older with vasoocclusive crises associated with sickle cell disease (SCD) and if the conditions indicated below are met. Adakveo is considered investigational in patients less than 16 years of age and for all other indications.

Initial Coverage (12 months):
 
Documented SCD genotype.
 
Age ≥16 years.
 
≥2 VOC requiring healthcare visits in the past 12 months despite HU, or HU contraindicated/not tolerated.
 
Prescriber: hematologist or SCD specialist.
 
Use per FDA dosing schedule.
 
 
Reauthorization (12 months):
 
Evidence of clinical benefit: ↓ VOC frequency/severity and adherence.
 
 
Non-Coverage:
 
Patients <16 years.
 
No recent VOC requiring healthcare.
 
Chronic transfusion prophylaxis.
 
Use outside FDA label without compendia support.

Policy Guidelines

Required documents: genotype confirmation, VOC history, HU history, infusion plan.
 
Lab interference: platelet clumping—repeat counts or use citrate tubes.
 
Clinical guideline: NIH/NHLBI (2024) includes Adakveo® as option in recurrent VOC.

DOSAGE/ADMINISTRATION

(PI 06/2024)
 
5 mg/kg IV at Week 0, Week 2, then every 4 weeks.
 
Infusion: 30 minutes, 0.2 μm in-line filter, 100 mL NaCl 0.9% or D5W.
 
Missed dose management: ≤2 weeks late → resume; >2 weeks → reset schedule.
 
Monitor IRR; avoid corticosteroids unless indicated.

REQUIRED MEDICAL INFORMATION

Initial Approval Criteria

a)    Diagnosis: to reduce the frequency of vasoocclusive crises (VOCs) in patients with sickle cell disease, and patient is at least 16 years of age AND
b.    Medication is prescribed by a physician specializing in sickle cell disease (e.g., hematologist) AND
c.    Member has been diagnosed with sickle cell disease (any genotype, including HbSS, HbSC, HbS/beta0-thalassemia, HbS/beta+-thalassemia, etc) AND
d.    Member has experienced at least 2 painful episodes of vaso-occlusive crises (VOCs) in the previous 12 months as determined by medical documentation AND
e.    One of the following applies:

• Member will be receiving hydroxyurea concomitantly with Adakveo OR
• Member has had an inadequate treatment response to at least a 3-month trial with a maximum tolerated dose of hydroxyurea
• Member is not a candidate for hydroxyurea treatment due to previously experienced hematologic toxicity reactions with this medication.

b)    Document: Patient’s actual body weight (recommended dose is 5 mg/kg).

Renewal Criteria for Approval

a)    The patient has disease improvement while using the requested agent, as demonstrated by a decrease in the frequency of vasoocclusive crisis (VOC) events [medical record documentation required]; AND
b)    The patient will NOT be using crizanlizumab-tmca (Adakveo® ) in combination with voxelotor (Oxbryta™) [medical record documentation required]; AND
c)    The prescriber is a specialist in the area of the patient’s diagnosis (e.g., hematologist, SCD specialist) or has consulted with a specialist in the area of the patient’s diagnosis [medical record documentation required]

EXCLUSION CRITERIA

Hypersensitivity to crizanlizumab.
 
Age <16 years.
 
No clinically significant VOC.
 
Chronic transfusion prophylaxis.

BENEFIT APPLICATION

Benefits are determined by the group contract, member benefit booklet, and/or individual subscriber certificate in effect at the time services were rendered. Benefit products or negotiated coverages may have all or some of the services discussed in this medical policy excluded from their coverage.

OTHER CRITERIA

N/A

Rational

Population Reference No. 1 Policy Statement

SUSTAIN: VOC reduction, improved hospitalization outcomes.
 
STAND: No superiority vs placebo.
 
Real-world data: Mixed; some ↓ VOC/↓ opioid use.

References

1. Product Information: ADAKVEO(R) intravenous injection, crizanlizumab-tmca intravenous injection. Novartis Pharmaceuticals Corporation (per FDA), East Hanover, NJ, 2021.

2. Crizanlizumab-tmca. In: DRUGDEX [database on the Internet]. Greenwood Village (CO): IBM Corporation; 2022 [cited 2022 Apr 16]. Available from: www.micromedexsolutions.com. Subscription required to view.

3. Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017;376:429-39.

4. FDA PI, Adakveo®, June 2024.
 
5. SUSTAIN (Ataga et al, NEJM 2017).
 
6. STAND (NCT03814746, 2023).
  
7. NIH/NHLBI SCD Guidelines, 2024.

Codes

Applicable Modifiers

Some modifires

Policy History