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

Coverage Summary & Scope

Defines UnitedHealthcare Commercial and Individual Exchange medical benefit coverage criteria, continuation criteria, exclusions, applicable codes, and administrative details for Adakveo (crizanlizumab-tmca). Applies to adults and pediatric patients aged 16 years and older with sickle cell disease and references FDA labeling for dosing and duration; coverage is for reduction in frequency of vasoocclusive crises and is provided under UnitedHealthcare Commercial and Individual Exchange benefit plans.

Initial Therapy (Medically Necessary Criteria)

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 (includes but not limited to HbSS, HbSC, sickle beta 0 thalassemia, 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 VOCs in the previous 12 months
  Definition: VOC = acute episode of pain requiring medical facility visit and opioids/NSAIDs; includes ACS, hepatic/splenic sequestration, priapism when requiring facility visit.
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 limits for initial approvals

ALL of the following

Initial dosing is in accordance with the United States Food and Drug Administration approved labeling
Refer to FDA labeling for dosing schedule (e.g., IV 5 mg/kg at weeks 0, 2, then every 4 weeks).
Initial authorization will be for no more than 12 months<= 12 months

Continuation Therapy (Reauthorization Criteria)

Criteria required for continuation (reauthorization) of Adakveo

ALL of the following

Diagnosis of a sickle cell disease (includes but not limited to HbSS, HbSC, sickle beta 0 thalassemia, 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
Document clinical response (reduction in VOC frequency and/or severity vs pretreatment baseline).
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< 16 years
Safety and efficacy not established in <16 years per FDA; consider not medically necessary.
Myelofibrosis

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 when requiring a facility visit.

Provider Actions & Administrative Requirements

Prior Authorization

Prior authorization required for initial and continuation therapy

Adakveo requires prior authorization for both initial and continuation therapy. Initial and reauthorization approvals are limited to no more than 12 months. Dosing (initial and maintenance) must follow the United States Food and Drug Administration–approved labeling, and prescriptions should be by or in consultation with a hematologist or specialist experienced in sickle cell disease.

J0791

Documentation Required

Document hydroxyurea status and VOC history

Document the patient’s current hydroxyurea status: either current hydroxyurea therapy, a history of treatment failure/intolerance/contraindication to hydroxyurea, or a provider attestation that hydroxyurea is not appropriate based on genotype. Also document the number of vaso-occlusive crises (VOCs) in the prior 12 months or that the patient is transitioning from voxelotor (Oxbryta).

Applicable Codes

J-code for drug billingHCPCSCovered

J0791

Injection, crizanlizumab-tmca, 5 mg

Applicable 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

Clinical Evidence

36% vs 17%Proportion with no VOCs (crizanlizumab 5 mg/kg vs placebo)

36%No VOCs on crizanlizumab 5 mg/kg

The SUSTAIN trial demonstrated that high‑dose crizanlizumab (5 mg/kg) reduced the median annual rate of VOCs versus placebo (1.63 vs 2.98; 45.3% lower, p=0.01) and increased the proportion of patients with no VOCs (36% vs 17%). Reductions were observed across genotypes and both with and without concomitant hydroxyurea. Crizanlizumab is a P‑selectin monoclonal antibody that blocks P‑selectin interactions on activated endothelium and platelets, preventing cell–cell interactions that contribute to vaso‑occlusion.

Background & Definitions

Adakveo (crizanlizumab‑tmca) is a humanized IgG2 kappa monoclonal antibody that binds to P‑selectin and blocks interactions with ligands including PSGL‑1, and can dissociate preformed P‑selectin/PSGL‑1 complexes; binding blocks interactions between endothelial cells, platelets, red blood cells, and leukocytes. Its efficacy was evaluated in the randomized, placebo‑controlled SUSTAIN trial in patients with sickle cell disease of various genotypes; the FDA indication is to reduce the frequency of VOCs in adults and pediatric patients aged 16 years and older. Initial and maintenance dosing should follow FDA‑approved labeling.

Revision History

05/01/2026template_updateLatest

Transferred content to a shared policy template that applies to both UnitedHealthcare Commercial and Individual Exchange benefit plans and added an Application section indicating applicability to Commercial and Individual Exchange plans; archived previous policy versions 2025D0085K and IEXD0085.10. (Template update; non-material change)