Adakveo® (Crizanlizumab-Tmca) – Commercial and Individual Exchange Medical Benefit Drug Policyopen_in_new

Coverage Summary

Coverage summary: This policy (Policy Number 2026D0085L, Status: CURRENT, Effective Date: May 1, 2026, Last Review: May 1, 2026) defines UnitedHealthcare Commercial and Individual Exchange medical benefit coverage for Adakveo (crizanlizumab-tmca). The drug is covered with criteria for reducing the frequency of vasoocclusive crises (VOCs) in patients with sickle cell disease who meet all specified initial and continuation criteria, including a minimum age of 16 years and other clinical and treatment-related requirements (see policy for full criteria).

FDA indication: Reduce frequency of VOCs in patients with sickle cell disease aged 16 years and older.

Initial Therapy

Covered when ALL of the following are met:

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 beta 0 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>= 2 in the previous 12 months
  VOC threshold
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

Initial Authorization Limits

Operational conditions for initial approval

ALL of the following

Initial dosing is in accordance with the United States Food and Drug Administration approved labeling
Dosing per FDA labeling
Initial authorization will be for no more than 12 months<= 12 months
Initial authorization length

Continuation Therapy (Reauthorization)

Covered 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 beta 0 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
Documented clinical benefit required for reauthorization
Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy
Patient is not receiving concomitant Oxbryta (voxelotor) therapy

Not Medically Necessary / Not Proven

Adakveo is considered not proven or not medically necessary for:

ANY of the following

Pediatric patients less than 16 years of age with sickle cell disease< 16 years
Excluded — safety and efficacy not established
Myelofibrosis
Excluded indication

Applicable Codes

Drug J-code (procedure code)HCPCSCovered

J0791

Injection, crizanlizumab-tmca, 5 mg

Relevant Diagnosis Codes (sickle cell disease)ICD-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

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Provider Actions / Authorization & Documentation

Prior Authorization

Prior authorization required

Initial and continuation therapy require prior authorization. Initial authorization will be for no more than 12 months and reauthorization will be for no more than 12 months. Adakveo must be prescribed by, or in consultation with, a hematologist or other specialist with expertise in sickle cell disease. Applicable procedure code: J0791 (Injection, crizanlizumab-tmca, 5 mg).

Documentation Required

Document hydroxyurea status or contraindication

Provider must document one of the following: current hydroxyurea therapy; history of treatment failure, intolerance, or contraindication to hydroxyurea; or provider attestation that the patient is not an appropriate candidate for hydroxyurea based on the patient's genotype.

Clinical Evidence

45.3%SUSTAIN primary outcome: reduction in median annual VOC rate vs placebo

1.63 vs 2.98Median annual VOC rate: crizanlizumab 5 mg/kg vs placebo

16+FDA indication: patients aged 16 years and older

Background and mechanism: Adakveo (crizanlizumab-tmca) is a humanized monoclonal antibody that binds to P-selectin, blocking its interactions (including with PSGL-1) and thereby inhibiting interactions among endothelial cells, platelets, red blood cells, and leukocytes.

SUSTAIN trial headline: In the randomized SUSTAIN trial, patients receiving crizanlizumab 5 mg/kg had a lower median annual VOC rate (1.63) versus placebo (2.98), representing a 45.3% reduction (p=0.01).

FDA indication for context: Reduce frequency of VOCs in patients with sickle cell disease aged 16 years and older.

Background & Definitions

Adakveo description and target population: Adakveo (crizanlizumab-tmca) is a humanized IgG2 kappa monoclonal antibody that binds to P-selectin and blocks P-selectin–mediated cellular interactions; it is indicated to reduce the frequency of vasoocclusive crises in patients with sickle cell disease aged 16 years and older. The policy targets patients with sickle cell disease (various genotypes) who meet specified clinical criteria for initial or continuation therapy.

Definitions: Vasoocclusive crisis (VOC) — An acute episode of pain with no cause other than a vaso-occlusive event requiring a medical facility visit and oral or parenteral opioids, or parenteral NSAIDs; includes acute chest syndrome, hepatic sequestration, splenic sequestration, and priapism requiring a medical facility visit.

Revision History

2026-05-01template_updateLatest

Transferred content to shared policy template that applies to both UnitedHealthcare Commercial and Individual Exchange benefit plans; added Application section.

2026-05-01archived_versionsLatest

Archived previous policy versions 2025D0085K and IEXD0085.10; template update.