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    OpenPayerPolicy UpdatesHematology/OncologyBlue Cross Blue Shield Iowa mitapivat Coverage Upd…
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    CurrentBlue Cross Blue Shield - IowaPolicy 05.04.62

    Aqvesme (mitapivat) and Pyrukynd (mitapivat) — Coverage Criteria for Thalassemia and Pyruvate Kinase Deficiency

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    Policy governs prior authorization, coverage criteria, and quantity limits for mitapivat (Aqvesme and Pyrukynd) for treatment of hemolytic anemia due to alpha- or beta-thalassemia and pyruvate kinase (PK) deficiency for Wellmark / Blue Cross Blue Shield members.

    Policy Summary
    PayerBlue Cross Blue Shield - Iowa
    PolicyAqvesme (mitapivat) and Pyrukynd (mitapivat) — Coverage Criteria for Thalassemia and Pyruvate Kinase Deficiency
    Policy CodePolicy 05.04.62
    Change TypeNo material change
    Effective DateMay 15, 2026
    Last ReviewApr 1, 2026
    Next Review Date
    Key ActionSubmit prior authorization with diagnostic confirmation, transfusion history, and baseline labs to support approval.
    SourceLink
    POLICY UPDATE CHANGES

    No material clinical or coverage changes in this revision.

    2distinct indications

    OpenPayer is powered by Trek Health's payer performance platform. Trek continuously ingests, validates, and normalizes Transparency in Coverage data alongside payer policies and other commercial payer data to create a structured payer intelligence foundation. OpenPayer uses this foundation to deliver personalized search results, dynamically generated policy pages, and tailored policy monitoring based on each user's payers, specialties, billing codes, and areas of interest. The same intelligence powers broader payer performance workflows, including reimbursement benchmarking, contract evaluation, payer negotiations, and financial decision-making.

    ≥1.5 g/dL
    hemoglobin response
    6 monthsapproval duration
    6 units/24wthalassemia transfusion threshold
    ≥6/52w or Hb≤10PK deficiency transfusion/Hb

    Coverage Criteria for Mitapivat (Aqvesme, Pyrukynd)

    Initial Therapy - Thalassemia

    Aqvesme (mitapivat) covered when ALL of the following are met for alpha- or beta-thalassemia:

    Thalassemia initial coverage criteria: 1) Symptomatic anemia with pretreatment/pretransfusion hemoglobin ≤ 11 g/dL; 2) Diagnosis of thalassemia (β-thalassemia with or without α-globin gene mutations, HbE/β-thalassemia, or α-thalassemia/HbH disease) confirmed by hemoglobin electrophoresis/HPLC OR molecular genetic testing; 3) Member does not have ANY of: homozygous or heterozygous sickle hemoglobin (HbS), homozygous or heterozygous hemoglobin C (HbC), or hepatic cirrhosis (Child‑Pugh A, B or C); 4) For non‑transfusion‑dependent β‑thalassemia: inadequate response, intolerance, or contraindication to compliant use of hydroxyurea OR genetic variant not anticipated to benefit from hydroxyurea; 5) For transfusion‑dependent β‑thalassemia: all of the following—A) inadequate response, intolerance, or contraindication to Reblozyl (luspatercept-aamt); B) mitapivat will not be used in combination with Reblozyl; C) history of requiring ≥ 6 RBC units transfused in the prior 24 weeks; 6) Prescribed by or in consultation with a hematologist or thalassemia specialist; 7) Age ≥ 18 years.

    Exact nested logic preserved from policy

    Continuation Therapy - Thalassemia

    Aqvesme continued treatment covered when ALL of the following are met:

    Thalassemia continuation criteria: 1) Member has achieved or maintained a positive clinical response defined by ONE of: increase in hemoglobin ≥1.5 g/dL from baseline OR reduction in blood transfusions from baseline; 2) Member has not experienced unacceptable toxicity (e.g., new or worsening jaundice or ALT ≥10× baseline); 3) Prescribed by or in consultation with a hematologist or specialist in the treatment of alpha‑ or beta‑thalassemia.

    Continuation approval duration is 6 months per policy

    Initial Therapy - PK deficiency

    Pyrukynd (mitapivat) covered when ALL of the following are met for pyruvate kinase (PK) deficiency:

    PK deficiency initial coverage criteria: 1) Member meets at least one: A) documented deficiency of PK enzyme activity by enzyme assay OR B) presence of ≥2 mutant alleles in the PKLR gene with at least one missense mutation; 2) Member is not homozygous for the R479H mutation AND does not have two non‑missense variants without another missense mutation in PKLR; 3) Member meets at least one: A) history of ≥6 blood transfusion episodes in the past 52 weeks OR B) hemoglobin ≤ 10.0 g/dL; 4) Receiving concomitant oral folic acid ≥ 0.8 mg daily; 5) Prescribed by or in consultation with a hematologist; 6) Age ≥ 18 years.

    All listed conditions required as written

    Continuation Therapy - PK deficiency

    Pyrukynd continued treatment covered when ALL of the following are met:

    PK deficiency continuation criteria: 1) Member has achieved or maintained a positive clinical response defined by ONE of: increase in hemoglobin ≥1.5 g/dL from baseline OR reduction in blood transfusions from baseline; 2) Member will continue concomitant oral folic acid ≥ 0.8 mg daily; 3) Prescribed by or in consultation with a hematologist.

    Continuation approval duration is 6 months per policy

    Coverage for Aqvesme (mitapivat) excludes members with certain hemoglobinopathies and advanced liver disease. Specifically, members who are homozygous or heterozygous for sickle hemoglobin (HbS) or hemoglobin C (HbC) are excluded. Members with hepatic cirrhosis of any Child‑Pugh class (A, B, or C) are also excluded from therapy. These exclusions apply to initial and continuation determinations as stated in the policy.

    Aqvesme and Pyrukynd (mitapivat) are considered not medically necessary for members who do not meet the specific coverage criteria described in this policy. Requests that lack the required diagnostic confirmation, transfusion history, baseline laboratory data, or documentation of prior therapy/response as outlined in the criteria may be denied.

    Procedures, Codes, and Thresholds

    Procedures and billing codesmixed
    No codes listed
    Transfusion or hemoglobin thresholds — Thalassemia and PK deficiency
    Thalassemia — transfusion-dependent thresholdHistory of requiring at least 6 RBC units transfused in the previous 24 weeks
    Thalassemia — symptomatic anemia thresholdPretreatment or pretransfusion hemoglobin ≤ 11 g/dL
    PK deficiency — transfusion history thresholdHistory of a minimum of 6 blood transfusion episodes in the past 52 weeks
    PK deficiency — hemoglobin thresholdHemoglobin concentration ≤ 10.0 g/dL

    Provider Actions and Prior Authorization Requirements

    Prior Authorization

    Prior authorization required

    Prior authorization is required for requests for Aqvesme or Pyrukynd. Submit clinical documentation (diagnostic test results, transfusion history, and baseline labs) with the initial prior authorization request to support medical necessity.

    Step Therapy

    Step therapy / prior agents required for thalassemia

    Step-therapy requirements vary by thalassemia subtype: non‑transfusion-dependent β‑thalassemia requires trial and inadequate response/intolerance/contraindication to hydroxyurea (or documented genetic variant unlikely to benefit); transfusion-dependent β‑thalassemia requires prior inadequate response/intolerance/contraindication to luspatercept and mitapivat must not be used with luspatercept.

    • Non‑transfusion-dependent β‑thalassemia: inadequate response/intolerance/contraindication to hydroxyurea OR genetic variant not anticipated to benefit
    • Transfusion‑dependent β‑thalassemia: inadequate response/intolerance/contraindication to Reblozyl (luspatercept-aamt) AND mitapivat will not be used in combination with Reblozyl

    Definitions and Clinical Response

    Positive clinical response — policy definition
    Definition of positive clinical responseIncrease in hemoglobin of at least 1.5 g/dL from baseline OR reduction in blood transfusions from baseline
    Use for continuation decisions (thalassemia)Continuation approved when member has achieved or maintained the positive clinical response
    Use for continuation decisions (PK deficiency)Continuation approved when member has achieved or maintained the positive clinical response; concomitant folic acid (≥0.8 mg daily) must be continued

    Background

    Mitapivat (marketed as Aqvesme and Pyrukynd) is an oral activator of pyruvate kinase indicated for adults with hemolytic anemia from alpha‑ or beta‑thalassemia (Aqvesme) and for hemolytic anemia due to pyruvate kinase deficiency (Pyrukynd). Coverage decisions align with FDA‑labeled indications and require diagnostic confirmation, baseline labs, and specialist prescribing; authorizations are issued for 6 months when criteria are met.

    Policy Summary
    PayerBlue Cross Blue Shield - Iowa
    PolicyAqvesme (mitapivat) and Pyrukynd (mitapivat) — Coverage Criteria for Thalassemia and Pyruvate Kinase Deficiency
    Policy CodePolicy 05.04.62
    Change TypeNo material change
    Effective DateMay 15, 2026
    Last ReviewApr 1, 2026
    Next Review Date
    Key ActionSubmit prior authorization with diagnostic confirmation, transfusion history, and baseline labs to support approval.
    SourceLink
    On This Page
    Coverage Criteria for Mitapivat (Aqvesme, Pyrukynd)Procedures, Codes, and ThresholdsProvider Actions and Prior Authorization RequirementsDefinitions and Clinical ResponseBackground
    Documentation Required

    Required documentation for prior authorization (initial requests)

    Initial prior authorization requests must include chart notes or medical records documenting transfusion history and pretreatment/pretransfusion hemoglobin, diagnostic confirmation, and baseline liver tests (for thalassemia) or genetic/enzyme testing (for PK deficiency).

    • Thalassemia initial: blood transfusion history and pretreatment/pretransfusion Hgb; hemoglobin electrophoresis or HPLC OR molecular genetic testing; baseline liver function tests (ALT, AST, alkaline phosphatase, total bilirubin with fractionation)
    • PK deficiency initial: enzyme assay demonstrating PK deficiency OR genetic testing with ≥2 PKLR mutant alleles (≥1 missense); documentation that member is not homozygous for R479H and does not have two non‑missense variants without another missense mutation; blood transfusion history or hemoglobin levels
    Documentation Required

    Documentation required for continuation/renewal

    For continuation/renewal, submit chart notes documenting a positive clinical response (either an increase in hemoglobin of at least 1.5 g/dL from baseline or a reduction in blood transfusions) and absence of unacceptable toxicity; for PK deficiency also document ongoing folic acid ≥0.8 mg daily.

    • Evidence of positive clinical response: Hgb increase ≥1.5 g/dL from baseline OR reduction in blood transfusions from baseline
    • Document absence of unacceptable toxicity
    • PK deficiency: documentation of continued concomitant folic acid ≥0.8 mg orally daily
    Denial Risk

    Denial risk if coverage criteria not met

    Requests may be denied as not medically necessary if members do not meet the policy's specified coverage criteria for the indicated diagnosis.