Adakveo Crizanlizumab Tmca Oh Cs

Coverage Summary & Rationale

Coverage stance: Covered with criteria for Adakveo (crizanlizumab-tmca) to reduce vasoocclusive crises (VOCs) in patients aged 16 years and older with sickle cell disease, per the policy summary and FDA indication. Effective date: July 1, 2025. Scope: UnitedHealthcare Medical Benefit Drug Policy applicable only to Ohio (defines initial and continuation criteria, exclusions, codes, prescribing requirements, dosing per FDA labeling, and authorization durations).

Coverage Rationale / Initial Therapy (Medically Necessary)

Adakveo is proven and/or medically necessary to reduce the frequency of vasoocclusive crises in patients with sickle cell disease who meet ALL of the following criteria:

ALL of the following

Patient is 16 years of age or older
Diagnosis of a sickle cell disease, including, but not limited to, homozygous hemoglobin S (HbSS), sickle hemoglobin C disease (HbSC), sickle beta0 thalassemia, and sickle beta+ thalassemia
ONE of
- Patient is transitioning from treatment with Oxbryta (voxelotor) to Adakveo
- Patient has previously experienced 2 or more sickle cell-related vasoocclusive crises within the previous 12 months
ONE of
- Patient is currently receiving hydroxyurea therapy
- Patient has a history of treatment failure, intolerance, or contraindication to hydroxyurea therapy
- Provider attests that the patient is not an appropriate candidate for hydroxyurea based on the patient's genotype (i.e., the patient does not have homozygous hemoglobin SS (HbSS) or sickle beta 0 thalassemia)
Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy
Patient is not receiving concomitant Oxbryta (voxelotor) therapy
Adakveo is prescribed by, or in consultation with, a hematologist, or other specialist with expertise in the diagnosis and management of sickle cell disease
Adakveo initial dosing is in accordance with the United States Food and Drug Administration approved labeling
Operational: initial authorization will be for no more than 12 months

ref(

Continuation of Therapy (Reauthorization)

Continuation of Therapy (Reauthorization Criteria)

Reauthorization will be granted when ALL of the following are met:

ALL of the following

Diagnosis of a sickle cell disease, including, but not limited to, homozygous hemoglobin S (HbSS), sickle hemoglobin C disease (HbSC), sickle beta0 thalassemia, and sickle beta+ thalassemia
Patient has experienced a reduction in sickle cell-related vasoocclusive crises and/or a decrease in severity of sickle cell-related vasoocclusive crises from pretreatment baseline while on Adakveo
Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy
Patient is not receiving concomitant Oxbryta (voxelotor) therapy

Not Medically Necessary / Exclusions

Not Medically Necessary / Not Proven

Adakveo is not proven or medically necessary for the following:

ANY of the following

Pediatric patients less than 16 years of age with sickle cell disease
Myelofibrosis

ref(

Applicable Codes

Drug HCPCSHCPCSCovered

J0791

Injection, crizanlizumab-tmca, 5 mg

Relevant Diagnosis Codes (Sickle Cell Disease and Variants) - informationalICD-10

D57.0	Hb-SS disease with crisis
D57.00	Hb-SS disease with crisis, unspecified
D57.01	Hb-SS disease with acute chest syndrome
D57.02	Hb-SS disease with splenic sequestration
D57.03	Hb-SS disease with cerebral vascular involvement
D57.04	Hb-SS disease with dactylitis
D57.09	Hb-SS disease with crisis with other specified complication
D57.1	Sickle-cell disease without crisis
D57.2	Sickle-cell/Hb-C disease
D57.20	Sickle-cell/Hb-C disease without crisis

1–10 of 52

1/6

Prior Authorization, Documentation & Billing Requirements

Prior Authorization

Initial prior authorization requirement and duration

Initial prior authorization (PA) is required for J0791 (Injection, crizanlizumab-tmca, 5 mg). Initial authorization is limited to no more than 12 months and must document that all initial therapy criteria are met, including: patient age (≥16 years); diagnosis of a sickle cell disease; prior VOC frequency or transition from voxelotor (Oxbryta); hydroxyurea status, including provider attestation of genotype-based inappropriateness when claimed (i.e., patient does not have HbSS or sickle beta0 thalassemia) or documentation of hydroxyurea use, intolerance, contraindication, or treatment failure when applicable; absence of concomitant chronic prophylactic blood transfusion; absence of concomitant voxelotor; and that Adakveo is prescribed by, or in consultation with, a hematologist or other relevant specialist. Initial and maintenance dosing must follow FDA-approved labeling.

Affected code: J0791
Initial authorization duration: up to 12 months
Required documentation: age, diagnosis, VOC history or transition from voxelotor, hydroxyurea status (including genotype attestation when claimed), absence of concomitant chronic prophylactic transfusion and voxelotor, specialist prescribing/consultation
Dosing per FDA label

Key Clinical Evidence

45.3%Lower median annual VOC rate vs placebo (1.63 vs 2.98)

4.1 vs 1.4Median time to first VOC (months) — crizanlizumab vs placebo

16+FDA indication: adults and pediatric patients aged 16 years and older

Evidence summary: The SUSTAIN randomized, placebo-controlled trial evaluated crizanlizumab 5 mg/kg versus placebo over 52 weeks in patients with sickle cell disease and a history of 2–10 VOCs in the prior 12 months. Patients receiving crizanlizumab 5 mg/kg had a lower median annual VOC rate (1.63 vs 2.98, a 45.3% lower rate) and an increased median time to first VOC (4.1 vs 1.4 months). Reductions in VOC frequency were observed across genotypes and regardless of hydroxyurea use. The FDA indication is to reduce frequency of VOCs in adults and pediatric patients aged 16 years and older with sickle cell disease. The safety and efficacy of Adakveo in pediatric patients <16 years have not been established.

Background & Definitions

Background: Adakveo (crizanlizumab-tmca) is a humanized IgG2 kappa monoclonal antibody that binds to P-selectin, blocking interactions with ligands (including PSGL-1) and inhibiting cell interactions that contribute to vaso-occlusion. It is indicated to reduce vasoocclusive crises in patients aged 16 years and older with sickle cell disease. The safety and efficacy in pediatric patients <16 years of age have not been established. Scope/high-level payer applicability: This UnitedHealthcare Medical Benefit Drug Policy applies only to Ohio and defines initial and continuation coverage criteria, exclusions, applicable codes, prescribing requirements, dosing alignment with FDA labeling, and authorization durations.

Definition: Vasoocclusive crisis (VOC) — An acute episode of pain related to vaso-occlusion requiring a medical facility visit and treatment with oral or parenteral opioids or parenteral NSAIDs; includes acute chest syndrome, hepatic sequestration, splenic sequestration, and priapism when requiring a medical facility visit.

Policy Revision History

07/01/2025material_changeLatest

Revised coverage criteria to permit genotype-based attestation as an alternative to documented hydroxyurea failure/intolerance/contraindication: coverage allowed when the provider attests the patient is not an appropriate candidate for hydroxyurea based on genotype (i.e., the patient does not have homozygous hemoglobin SS (HbSS) or sickle beta0 thalassemia).

07/01/2025supporting_information_updateLatest

Updated References section to reflect the most current information and archived previous policy version CSOH2025D0085.D.