EmblemHealth Clotting Disorder Coverage Update

Coverage Criteria

Initial Therapy — Covered when ALL of the following product-specific conditions are met

Covered when ALL of the following product-specific conditions are met:

Criteria I (AlphaNine SD, Alprolix, BeneFIX, Idelvion, Profilnine SD, Rixubis, IXINITY, Rebinyn): For Hemophilia B: A. Congenital factor IX deficiency confirmed by blood coagulation testing; AND B. Used to treat at least ONE: i. Control and prevention of acute bleeding episodes (hemorrhage) OR ii. Routine prophylaxis to prevent or reduce frequency of bleeding episodes (applicable to Alprolix, BeneFIX, Idelvion, IXINITY, Rebinyn, and Rixubis only) OR iii. Perioperative management; AND C. Not used for induction of immune tolerance in members with Hemophilia (applies to Alprolix, Rixubis, Idelvion, IXINITY, Rebinyn, AlphaNine SD, Mononine and Benefix).

from Criteria I

Criteria III (Advate, Adynovate, Altuviiio, Eloctate, Hemofil M, Koate-DVI, Kogenate FS, Novoeight, Recombinate, Xyntha, NUWIQ, Afstyla, Kovaltry, Jivi, Esperoct): For Hemophilia A: A. Congenital factor VIII deficiency confirmed by blood coagulation testing; AND B. Used to treat at least ONE: i. Control and prevention of acute bleeding episodes (hemorrhage) OR ii. Routine prophylaxis to prevent or reduce the frequency of bleeding episodes OR iii. Perioperative management.

from Criteria III

Von Willebrand disease (Alphanate, Humate-P specifics): A. Von Willebrand disease confirmed by blood coagulation and von Willebrand factor testing; AND B. Used as surgical bleeding prophylaxis in members with VWD in whom desmopressin is ineffective or contraindicated, OR to control episodes of acute bleeding (Humate-P only); AND C. Alphanate is not indicated for members with severe (type 3) VWD undergoing major surgery; AND D. HUMATE-P is not indicated for the prophylaxis of spontaneous bleeding episodes in VWD.

from chunk 11

Criteria IV (Feiba NF) - inhibitors: For members with inhibitors: A. Congenital factor VIII deficiency confirmed by blood coagulation testing; AND B. Confirmation that member has Factor VIII inhibitors; AND C. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Routine prophylaxis to prevent or reduce the frequency of episodic bleeding OR iii. Perioperative management.

Feiba NF also has separate Medicare LCD/NCD criteria referenced

Feiba NF - Hemophilia B with inhibitors: A. Congenital factor IX deficiency confirmed by blood coagulation testing; AND B. Confirmation that member has Factor IX inhibitors; AND C. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Routine prophylaxis to prevent or reduce the frequency of episodic bleeding OR iii. Perioperative management.

from chunk 15

Acquired Factor VIII inhibitors: A. Diagnosis confirmed by blood coagulation testing; AND B. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Perioperative management.

from chunk 16

Acquired Hemophilia (general): A. Diagnosis confirmed by blood coagulation testing; AND B. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Perioperative management.

from chunk 17

Acquired Factor IX inhibitors: A. Factor IX deficiency confirmed by blood coagulation testing; AND B. Confirmation that member has acquired Factor IX inhibitors; AND C. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Perioperative management.

from chunk 18

Congenital Factor VII deficiency: A. Factor VII deficiency confirmed by blood coagulation testing; AND B. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Perioperative management.

from chunk 19

Covered indications by specific bleeding disorder — Covered when ALL of the following for the specified disorder are met

Covered when ALL of the following for the specified disorder are met

Hemophilia B: A. Factor IX deficiency confirmed by blood coagulation testing; AND B. Confirmation that member has acquired Factor IX inhibitors (when applicable); AND C. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Perioperative management.

from chunk 18

Congenital Factor VII Deficiency: A. Factor VII deficiency confirmed by blood coagulation testing; AND B. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Perioperative management.

from chunk 19

Glanzmann's Thrombasthenia: A. Glanzmann's Thrombasthenia confirmed by blood coagulation testing; AND B. Used to treat at least ONE: i. Control and prevention of acute episodic bleeding (hemorrhage) OR ii. Perioperative management; AND C. Platelet transfusions are known or suspected to be ineffective or contraindicated.

from chunk 20

Renewal Criteria — Renewal coverage requires ALL of the following

Renewal coverage requires ALL of the following

Renewal safety: Absence of unacceptable drug-toxicity such as symptoms of allergic-anaphylactic reactions (e.g., anaphylaxis, dyspnea, rash), thromboembolic events (e.g., thromboembolism, pulmonary embolism), and development of neutralizing antibodies (inhibitors).

from chunk 30

Alphanate and HUMATE‑P have product‑specific indications for von Willebrand disease (VWD). VWD must be confirmed by blood coagulation testing and von Willebrand factor testing. These products are covered when used as surgical bleeding prophylaxis in members for whom desmopressin is ineffective or contraindicated. Humate‑P is also explicitly described as indicated to control episodes of acute bleeding. Additionally, Alphanate is not indicated for members with severe (type 3) VWD undergoing major surgery, and HUMATE‑P is not indicated for prophylaxis of spontaneous bleeding episodes in VWD.

Requests for hemophilia and related clotting‑factor medications are eligible for coverage only for the specific indications listed in this policy. Per the policy's limitations, these medications are considered investigational and not medically necessary for any indications other than those listed, and requests for unlisted uses may be denied.

The policy revision clarifies product‑specific therapy restrictions: Mononine is not to be used for induction of immune tolerance in members with hemophilia. This restriction applies to the listed products including Alprolix, Rixubis, Idelvion, IXINITY, Rebinyn, AlphaNine SD, Mononine, and BeneFIX.

Any use of hemophilia or clotting‑factor therapies that is not explicitly listed in the Covered Indications section of this policy is considered investigational and not medically necessary. Providers should include documentation that the requested use matches a listed indication; otherwise, the request may be denied.

Coding

Applicable HCPCS / J-codes (group 1)HCPCSCovered

J7170	Injection, emicizumab-kxwh, 0.5 mg.
J7175	Injection, factor x, (human), 1 i.u. (Coagadex).
J7179	Injection, von willebrand factor (recombinant), (vonvendi), 1 i.u. vwf:rco.
J7180	Injection, factor XIII (antihemophilic factor, human), 1 IU.
J7181	Injection, factor XIII A-subunit, (recombinant), per IU.
J7182	Injection, factor VIII, (antihemophilic factor, recombinant), (NovoEight), per IU.
J7183	Injection, von Willebrand factor complex (human), Wilate, 1 IU vWF:RCo.
J7185	Injection, factor VIII (antihemophilic factor, recombinant) (XYNTHA), per IU.
J7186	Injection, antihemophilic factor VIII/von Willebrand factor complex (human), per factor VIII i.u..
J7187	Injection, von Willebrand factor complex (Humate-P), per IU VWF:RCO.

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Applicable HCPCS / J-codes (group 2)HCPCSCovered

J7199	Hemophilia clotting factor, not otherwise classified.
J7200	Injection, factor IX, (antihemophilic factor, recombinant), Rixubis, per IU.
J7201	Injection, factor ix, fc fusion protein, (recombinant), Alprolix, 1 i.u..
J7202	Injection, factor ix, albumin fusion protein, (recombinant), Idelvion, 1 i.u..
J7203	Injection, factor ix (antihemophilic factor, recombinant), glycopegylated, Rebinyn, 1 i.u..
J7204	Injection, factor viii, antihemophilic factor (recombinant), glycopegylated-exei, per iu (Esperoct).
J7205	Injection, factor VIII Fc fusion protein (recombinant), per IU.
J7207	Injection, factor viii, (antihemophilic factor, recombinant), pegylated, 1 i.u..
J7208	Injection, factor viii (antihemophilic factor, recombinant) pegylated-aucl, Jivi, 1 i.u..
J7212	Intravenous Powder for Solution, Factor viia, (antihemophilic factor, recombinant)-jncw (sevenfact), 1 microgram.

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Infusion and administration CPT codesCPT

96365	Intravenous infusion; initial, up to 1 hour.
96366	Intravenous infusion; each additional hour.
96367	Intravenous infusion; additional sequential infusion of a new drug/substance, up to 1 hour.
96368	Intravenous infusion; concurrent infusion.
96369	Subcutaneous infusion; initial, up to 1 hour, including pump set-up.
96370	Subcutaneous infusion; each additional hour.
96371	Subcutaneous infusion; additional pump set-up with new site(s).
96372	Therapeutic/prophylactic/diagnostic injection; subcutaneous or intramuscular.
96373	Therapeutic/prophylactic/diagnostic injection; intra-arterial.
96375	Each additional sequential intravenous push of a new substance/drug.

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ICD-10 diagnosis codesICD-10

D66	Hereditary factor VIII deficiency.
D67	Hereditary factor IX deficiency.
D68.0	Von Willebrand's disease.
D68.1	Hereditary factor XI deficiency.
D68.2	Hereditary deficiency of other clotting factors.
D68.311	Acquired hemophilia.
D68.9	Coagulation defect, unspecified.
D69.1	Qualitative platelet defects.
R58	Hemorrhage, not elsewhere classified.

Applicable Procedure CodesCPT

96375	Therapeutic, prophylactic, or diagnostic injection; each additional sequential intravenous push of a new substance/drug.
96376	Therapeutic, prophylactic, or diagnostic injection; each additional sequential intravenous push of the same substance/drug provided in a facility.
96377	Application of on-body injector (includes cannula insertion) for timed subcutaneous injection.
96379	Unlisted therapeutic, prophylactic, or diagnostic intravenous or intra-arterial injection or infusion.
99601	Home infusion/specialty drug administration, per visit (up to 2 hours).
99602	Home infusion/specialty drug administration, per visit (up to 2 hours); each additional hour.

Applicable J-codes addedHCPCS

J7214

Injection, factor viii/von willebrand factor complex, recombinant (altuviiio), per factor viii i.u.

ICD-10 DiagnosesICD-10

D66	Hereditary factor VIII deficiency.
D67	Hereditary factor IX deficiency.
D68.0	Von Willebrand's disease.
D68.1	Hereditary factor XI deficiency.
D68.2	Hereditary deficiency of other clotting factors.
D68.311	Acquired hemophilia.
D68.9	Coagulation defect, unspecified.
D69.1	Qualitative platelet defects.
R58	Hemorrhage, not elsewhere classified.

inv-15: Age threshold for Sevenfact in inhibitor patients

Sevenfact age requirementPatient is ≥ 12 years of age

Applicable diagnoses (context)Hemophilia A or B with inhibitors (congenital factor VIII or IX deficiency)

Diagnostic confirmation requiredDiagnosis of congenital factor VIII or IX deficiency must be confirmed by blood coagulation testing

Provider Actions & Requirements

Prior Authorization

Length of Authorization

Initial authorization period is six months.

Billing Rule

Procedure and J-code Submission Requirement

Use the applicable procedure and J-code(s) when submitting prior authorization requests. Examples of applicable J-codes include, but are not limited to: J7170, J7175, J7179, J7180–J7189, J7190, J7192–J7199, J7200–J7214, J3590, and infusion/administration codes (96365–96367, 96375–96379, 99601–99602). Include the specific J-code(s) that correspond to the product being requested (e.g., J7214 for Altuviiio).

Examples: J7214 (Altuviiio)
Include infusion/administration codes when applicable (e.g., 96365, 99601)

Background

This policy addresses both inherited and acquired bleeding disorders caused by deficiencies or dysfunction of clotting factors. Covered conditions include hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency), von Willebrand disease, congenital deficiencies of factors VII and XIII, acquired hemophilia and acquired inhibitors, Glanzmann thrombasthenia, and other congenital clotting‑factor deficiencies. Treatments addressed by the policy include factor replacement products, bypassing agents, and related biologics administered under the medical benefit. Diagnostic confirmation by blood coagulation testing (and von Willebrand factor testing where specified) is required for coverage determinations, and recent revisions add diagnosis confirmation language for certain products (for example, Sevenfact) and product‑specific restrictions reflected in the coverage criteria.

Definitions

inv-30: Hemophilia A — factor VIII deficiency definition

DefinitionHemophilia A = factor VIII (FVIII) deficiency; genetic disorder caused by missing or defective factor VIII

Diagnostic confirmationFactor VIII deficiency must be confirmed by blood coagulation testing for coverage

Common uses noted in policyRoutine prophylaxis to reduce frequency of bleeding episodes, on‑demand treatment and perioperative management

inv-31: Hemophilia B — factor IX deficiency definition

DefinitionHemophilia B = factor IX (FIX) deficiency; genetic disorder caused by missing or defective factor IX

Diagnostic confirmationFactor IX deficiency must be confirmed by blood coagulation testing for coverage

OpenPayer

Clotting Disorder Therapy

Coverage Criteria

Coding

Provider Actions & Requirements

Background

Definitions