Anthem Blue Cross California Agents for Hemophilia and von Willebrand Syndrome Form

Medical Drug Clinical Criteria Subject: Agents for Hemophilia A and von Willebrand Disease Document #: CC-0065 Status: Revised Table of contents Overview Clinical criteria Overview Publish Date: 12/18/2023 Last Review Date: 11/17/2023 Coding Document history References This document addresses select agents for hereditary or congenital hemophilia A, also called factor VIII (FVIII) deficiency or classic hemophilia and select agents for von Willebrand disease. Acquired hemophilia A (also called acquired factor VIII deficiency) is a rare autoimmune disorder, and not a congenital disease. Acquired hemophilia A requires individualized treatment, which is addressed by some of the agents in this document. This document does not address fibrin products, fibrin sealants and blood products provided by blood banks. Bypassing agents (i.e., NovoSeven RT, SevenFact, and FEIBA) for those who develop antibodies or inhibitors to factor products, and Stimate (desmopressin acetate) intranasal spray are discussed in separate documents. Factor replacement treatments can be created from blood products (human plasma-derived) and others that are manufactured (recombinant). Replacement therapy may be given on a routine, preventive basis which is also called prophylactic therapy. The infusion of factor replacements given to stop a bleeding episode is called on-demand or episodic therapy. While the World Federation of Hemophilia (WFH) (Srivastava 2020) does not place a preference between plasma-derived and recombinant products, the U.S. National Hemophilia Federation (NHF 2020) recommends recombinant over plasma-derived due the possibility of virus transmission. WFH states that the choice between the two classes of products must be made according to the availability, cost, and patient preferences. Products in this document include: • Anti-hemophilic factor (factor VIII) Human plasma derived o Hemofil-M, Koate-DVI • Anti-hemophilic factor (factor VIII) Recombinant o Advate, Afstyla, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, Xyntha/Xyntha Solofuse • Anti-hemophilic factor (factor VIII) – Long acting o Recombinant, Pegylated – Adynovate, o Recombinant, Pegylated damactocog alfa pegol – Jivi o Recombinant Fc Fusion Protein–Eloctate o Recombinant, Glycopegylated– Esperoct o Recombinant Anti-hemophilic factor Fc-VWF-XTEN Fusion Protein-ehtl – Altuviiio • Anti-hemophilic bispecific factor (Factor IXa- and Factor X-) o Hemlibra (emicizumab-kxwh) • Anti-hemophilic Factor VIII (Recombinant), Porcine Sequence o Obizur • Anti-hemophilic Factor VIII/von Willebrand Factor Complex o Alphanate, Humate-P, Wilate von Willebrand factor, Recombinant • o Vonvendi Hereditary hemophilia A is the most common type of hemophilia. Although it is usually inherited, about one third of cases are caused by spontaneous mutations. Hemophilia A is related to mutations in the gene coding for coagulation Factor VIII, and it is four times more common than hemophilia B (CDC 2014), the second most common hemophilia type. The U.S. National Hemophilia Foundation (NHF) and the World Federation of Hemophilia (Srivastava 2020) both note there is a relationship of bleeding severity to the clotting factor level. Both entities list “severe” hemophilia as a clotting factor level < 1 IU/dl or < 1% of normal. A “mild” bleeding severity is identified as a clotting factor level of 5-40 IU/dl or 5 to < 40% of normal. A bleeding episode for individuals with mild risk includes severe bleeding with major trauma or surgery. Individuals with 1-5 IU/dl or 1-5% of normal are considered “moderate” risk for occasional spontaneous bleeding and prolonged bleeding with minor trauma or surgery (Srivastava 2020). 1 Hemophilia severity: • Severe hemophilia – Severe hemophilia is defined as < 1 percent factor activity, which corresponds to < 1 IU/dL. • Moderate hemophilia – Moderate hemophilia is defined as a factor activity level ≥ 1 percent of normal and < 5 percent of normal, corresponding to ≥ 1 and < 5 IU/dL. • Mild hemophilia – Mild hemophilia is defined as a factor activity level ≥ 5 percent of normal and < 40 percent of normal (≥ 5 and < 40 IU/dL). World Federation of Hemophilia 2020 Guidelines for treatment of hemophilia state that prophylaxis prevents bleeding and joint destruction, and that prophylaxis should enable those with hemophilia to lead healthy and active lives. Moreover, the updated 2020 guidelines proposes that the definition of prophylaxis be based on outcomes rather than doses or timing of initiation, and treatment regimens that take into account the hemophilic phenotype of the individual in addition to factor levels. However, more studies are needed to determine if all individuals should remain on therapy as adults (that is, those with severe hemophilia vs. moderate or mild). The WFH 2020 guidelines have been endorsed by several societies worldwide, including the U.S. NHF. Short-term prophylaxis (of 4 to 8 weeks) may interrupt the bleeding cycle and benefit individuals with repeated bleeding into target joints. Prophylaxis does not reverse existing joint damage but reduces bleeding and may slow progression of joint damage. Prophylactic clotting factor administration is recommended prior to the individual engaging in activities with higher risk of injury. Randomized trials of prophylactic therapy of hemophilia have demonstrated a decreased incidence of arthropathy (Gringeri, 2011; Manco-Johnson, 2007). Von Willebrand disease (VWD) is the most common inherited bleeding disorder. It is caused by missing for defective von Willebrand factor (VWF), a clotting protein. Unlike hemophilia, which very rarely occurs in females, von Willebrand disease can occur equally in men and women. There are three main types of hereditary VWD – type 1 (most common), type 2, and type 3. The types are classified by their level of VWF in the blood or by the presence or behavior of the VWF chains. VWD can be caused by an autoimmune disorder or as a result of certain medications. This is called acquired von Willebrand disease. Those with VWD can experience frequent nosebleeds, easy bruising, and excessive bleeding after during and after surgical procedures. Women may have heavy and long lasting menstrual periods and hemorrhaging after childbirth (NHF). Hemlibra has a black box warning for thrombotic microangiopathy and thromboembolism, especially when administered with activated prothrombin complex concentrate (aPCC). Clinical Criteria When a drug is being reviewed for coverage under a member’s medical benefit plan or is otherwise subject to clinical review (including prior authorization), the following criteria will be used to determine whether the drug meets any applicable medical necessity requirements for the intended/prescribed purpose. Hemofil M, Koate/Koate-DVI (Factor VIII Human plasma-derived) Initial requests for Hemofil M or Koate/Koate-DVI (Factor VIII, human plasma-derived) may be approved if the following criteria are met: I. II. Individual has a diagnosis of hemophilia A (also called factor VIII deficiency or classic hemophilia); AND Individual is using for the treatment of bleeding episodes; OR III. IV. V. OR VI. VII. VIII. IX. Individual has a diagnosis of hemophilia A (also called factor VIII deficiency or classic hemophilia); AND Individual is using as routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND Individual has a diagnosis of severe hemophilia A (defined as less than 1 International Unit per deciliter [1IU/dL] or 1% endogenous Factor VIII) (NHF, Srivastava 2020); Individual has a diagnosis of hemophilia A (also called factor VIII deficiency or classic hemophilia); AND Individual is using as routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND Individual has a diagnosis of mild to moderate hemophilia A (defined as endogenous Factor VIII less than 40 IU/dL [less than 40%], but greater than or equal to 1 IU) (NHF, Srivastava 2020); AND Individual has one of the following (NHF, Srivastava 2020): A. One or more episodes of spontaneous bleeding into joint; OR B. One or more episodes of severe, life-threatening, or spontaneous bleeding as determined by the prescriber; OR C. Severe phenotype hemophilia determined by the individual’s risk factors that increase the risk of a clinically significant bleed, including but not limited to, participation in activities likely to cause injury/trauma, procoagulant and anticoagulant protein levels, comorbid conditions affecting functional ability and physical coordination, or history of a clinically significant bleed. Initial requests for Koate/Koate-DVI (Factor VIII, human plasma-derived) may be approved if the following criteria are met: I. II. Individual has a diagnosis of hemophilia A (also called factor VIII deficiency or classic hemophilia); AND Individual is using for peri-procedural management for surgical, invasive or interventional radiology procedures. 2 Continuation requests for Hemofil M or Koate/Koate-DVI (Factor VIII, human plasma-derived) may be approved if the following criteria are met: I. Individual has had a positive therapeutic response to treatment (for example, reduction in frequency and/or severity of bleeding episodes). Hemofil M, Koate/Koate-DVI (Factor VIII, human plasma-derived) may not be approved for the following: Individual is using for the treatment of von Willebrand disease (VWD); OR I. II. When the above criteria are not met and for all other indications. Advate, Afstyla, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, Xyntha/Xyntha Solofuse (Factor VIII Recombinant) Initial requests for Advate, Afstyla, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, or Xyntha/Xyntha Solofuse (Factor VIII recombinant) may be approved if the following criteria are met: I. II. Individual has a diagnosis of hemophilia A (also called factor VIII deficiency or classic hemophilia); AND Individual is using for one of the following: A. Treatment of bleeding episodes; OR B. Peri-procedural management for surgical, invasive or interventional radiology procedures; OR III. IV. V. VI. OR VII. VIII. IX. X. Individual has a diagnosis of von Willebrand disease; AND Individual is using for the treatment of bleeding episodes; AND Individual is using in combination with Vonvendi (recombinant von Willebrand factor complex); AND Individual has a baseline factor VIII level less than 40 IU/dL [less than 40%] or are unknown (Vonvendi 2018); Individual has a diagnosis of von Willebrand disease; AND Individual is using for peri-procedural management for surgical, invasive or interventional radiology procedures; AND Individual is using in combination with Vonvendi (recombinant von Willebrand factor complex); AND Individual has a baseline factor VIII level less than 30 IU/dL [less than 30%] or are unknown (Vonvendi 2018). Initial requests for Advate, Afstyla, Kogenate FS, Kovaltry, Novoeight, Nuwiq, or Xyntha/Xyntha Solofuse (Factor VIII recombinant) may be approved if the following criteria are met: I. II. Individual is using as routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND Individual has a diagnosis of severe hemophilia A (defined as less than 1 International Unit per deciliter [1IU/dL] or 1% endogenous Factor VIII) (NHF, Srivastava 2020); OR III. IV. V. Individual has a diagnosis of mild to moderate hemophilia A (defined as endogenous Factor VIII less than 40 IU/dL [less than 40%], but greater than or equal to 1 IU) (NHF, Srivastava 2020); AND Individual is using as routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND Individual has one of the following: A. One or more episodes of spontaneous bleeding into joint; OR B. One or more episodes of severe, life-threatening, spontaneous bleeding as determined by the prescriber ; OR C. Severe phenotype hemophilia determined by the individual’s risk factors that increase the risk of a clinically significant bleed, including but not limited to, participation in activities likely to cause injury/trauma, procoagulant and anticoagulant protein levels, comorbid conditions affecting functional ability and physical coordination, or history of a clinically significant bleed. Initial requests for Kogenate FS (Factor VIII recombinant) may be approved if the following criteria are met: I. II. III. Individual is 16 years of age or younger; AND Individual has a diagnosis of hemophilia A (also called factor VIII deficiency or classic hemophilia); AND Individual is using as routine prophylaxis to reduce the risk of joint damage in those without pre-existing joint damage. Initial requests for Recombinate (Factor VIII recombinant) may be approved if the following criteria are met: I. Individual is using for the treatment of acquired Factor VIII inhibitors not exceeding 10 Bethesda Unit (BU) per milliliter (mL). Continuation requests for Advate, Afstyla, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, or Xyntha/Xyntha Solofuse (Factor VIII recombinant) may be approved if the following criteria are met: 3 I. Individual has had a positive therapeutic response to treatment (for example, reduction in frequency and/or severity of bleeding episodes). Advate, Afstyla, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, or Xyntha/Xyntha Solofuse (Factor VIII recombinant) may not be approved for the following: Individual is using as monotherapy for the maintenance treatment of von Willebrand disease; OR I. II. When the above criteria are not met and for all other indications. Long Acting Agents: Adynovate (Factor VIII Long-Acting Recombinant, pegylated), Jivi (Factor VIII Recombinant, PEGylated damactocog alfa pegol), Eloctate (Factor VIII Recombinant Anti-hemophilic Factor Fc Fusion Protein), Esperoct (Factor VIII Recombinant, glycopegylated), or Factor VIII Recombinant Antihemophilic Factor FC-VWF-XTEN Fusion Protein (Altuviiio) Initial requests for Adynovate (Factor VIII Long-Acting Recombinant, pegylated), Jivi (Factor VIII Recombinant PEGylated damactocog alfa pegol), Eloctate (Factor VIII Recombinant Anti-hemophilic Factor Fc Fusion Protein), Esperoct (Factor VIII Recombinate, glycopegylated), or Altuviiio (Factor VIII Recombinant Antihemophilic Factor FC-VWF-XTEN Fusion Protein) may be approved if the following criteria are met: I. II. III. OR IV. V. Individual has a diagnosis of severe hemophilia A (defined as less than 1 International Unit per deciliter [1IU/dL] endogenous Factor VIII) (NHF, Srivastava 2020); AND Individual is using for one of the following: A. Treatment of acute bleeding episodes; OR B. Peri-procedural management for surgical, invasive or interventional radiology procedures; OR C. Routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND If using Jivi, individual is 12 years of age or older and has been previously treated with factor VIII; Individual has a diagnosis of mild to moderate hemophilia A (defined as endogenous Factor VIII less than 40 IU/dL [less than 40%], but greater than or equal to 1 IU) (NHF, Srivastava 2020); AND Individual is using for one of the following: A. Treatment of acute bleeding episodes; OR B. Peri-procedural management for surgical, invasive or interventional radiology procedures; OR C. Routine prophylaxis to prevent or reduce the frequency of bleeding episodes when one of the following: AND 1. 2. Individual has had one or more episodes of spontaneous bleeding into joint; OR Individual has had one or more episodes of severe, life-threatening, or spontaneous bleeding as determined by the prescriber; OR 3. Severe phenotype hemophilia determined by the individual’s risk factors that increase the risk of a clinically significant bleed, including but not limited to, participation in activities likely to cause injury/trauma, procoagulant and anticoagulant protein levels, comorbid conditions affecting functional ability and physical coordination, or history of a clinically significant bleed; AND If using Jivi, individual is 12 years of age or older and has been previously treated with factor VIII. VI. Continuation requests for Adynovate (Factor VIII Long-Acting Recombinant, pegylated), Jivi (Factor VIII Recombinant PEGylated damactocog alfa pegol), Eloctate (Factor VIII Recombinant Anti-hemophilic Factor Fc Fusion Protein), Esperoct (Factor VIII Recombinant, glycopegylated), or Altuviiio (Factor VIII Recombinant Antihemophilic Factor FC-VWF-XTEN Fusion Protein) may be approved if the following criteria are met: I. Individual has had a positive therapeutic response to treatment (for example, reduction in frequency and/or severity of bleeding episodes). Adynovate (Factor VIII Long-Acting Recombinant, pegylated), Jivi (Factor VIII Recombinant PEGylated damactocog alfa pegol), Eloctate (Factor VIII Recombinant Anti-hemophilic Factor Fc Fusion Protein), Esperoct (Factor VIII Recombinant, glycopegylated), or Altuviiio (Factor VIII Recombinant Antihemophilic Factor FC-VWF-XTEN Fusion Protein) may not be approved for the following: Individual is using for the treatment of von Willebrand disease; OR I. II. When the above criteria are not met and for all other indications. Hemlibra (emicizumab-kxwh) - Anti-hemophilic bispecific factor --- Factor IXa and Factor X Initial requests for Hemlibra (emicizumab-kxwh) may be approved if the following criteria are met: 4 I. II. III. OR IV. V. VI. VII. Individual has a diagnosis of severe hemophilia A (defined as less than 1 International Unit per deciliter [1IU/dL] endogenous Factor VIII) (NHF, Srivastava 2020); AND Individual is using for routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND Individual has one of the following: A. If switching from factor VIII agents, then individual will discontinue factor VIII agents being used for routine prophylaxis after the first week of Hemlibra initiation; OR If switching from bypassing agents (i.e., NovoSeven RT, SevenFact, FEIBA), then individual will discontinue bypassing agents being used for routine prophylaxis after 24 hours of Hemlibra initiation; B. Individual has a diagnosis of mild to moderate hemophilia A (defined as endogenous Factor VIII less than 40 IU/dL [less than 40%], but greater than or equal to 1 IU) (NHF, Srivastava 2020); AND Individual is using for routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND Individual has one of the following: A. One or more episodes of spontaneous bleeding into joint; OR B. One or more episodes of severe, life-threatening, or spontaneous bleeding as determined by the prescriber; OR C. Severe phenotype hemophilia determined by the individual’s risk factors that increase the risk of a clinically significant bleed, including but not limited to, participation in activities likely to cause injury/trauma, procoagulant and anticoagulant protein levels, comorbid conditions affecting functional ability and physical coordination, or history of a clinically significant bleed; AND Individual has one of the following: A. If switching from factor VIII agents, then individual will discontinue factor VIII agents being used for routine prophylaxis after the first week of Hemlibra initiation; OR If switching from bypassing agents, (i.e., NovoSeven RT, SevenFact, FEIBA), then individual will discontinue bypassing agents being used for routine prophylaxis after 24 hours of Hemlibra initiation. B. Continuation requests for Hemlibra (emicizumab-kxwh) may be approved if the following criteria are met: I. Individual has had a positive therapeutic response to treatment (for example, reduction in frequency and/or severity of bleeding episodes). Hemlibra (emicizumab) may not be approved when the above criteria are not met and for all other indications. Obizur (Factor VIII Recombinant, Porcine Sequence) Initial requests for Obizur (Recombinant, Porcine Sequence) may be approved if the following criteria are met: I. II. III. IV. Individual is 18 years of age or older; AND Individual has a diagnosis of acquired hemophilia A; AND Individual has baseline anti-porcine Factor VIII inhibitor titer less than or equal to 20 BU/mL; AND Individual is using for the treatment of bleeding episodes. Continuation requests for Obizur (Recombinant, Porcine Sequence) may be approved if the following criteria are met: I. Individual has had a positive therapeutic response to treatment (for example, reduction in frequency and/or severity of bleeding episodes). Obizur (Recombinant, Porcine Sequence) may not be approved for the following: Individual has a diagnosis of congenital hemophilia A with Factor VIII deficiency; OR I. Individual has a diagnosis of congenital hemophilia A with inhibitors; OR II. III. Individual has a diagnosis of von Willebrand disease; OR IV. When the above criteria are not met and for all other indications. Alphanate, Humate-P, Wilate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) Initial requests for Alphanate, Humate-P, or Wilate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) may be approved if the following criteria are met: I. II. Individual has a diagnosis of hemophilia A (also called factor VIII deficiency or classic hemophilia); AND Individual is using for the treatment of bleeding episodes; OR III. Individual has a diagnosis of severe hemophilia A (defined as less than 1 International Unit per deciliter [1IU/dL] endogenous Factor VIII) (NHF, Srivastava 2020); AND 5 IV. Individual is using for routine prophylaxis to prevent or reduce the frequency of bleeding episodes; OR V. VI. VII. Individual has a diagnosis of mild to moderate hemophilia A (defined as endogenous Factor VIII less than 40 IU/dL [less than 40%], but greater than or equal to 1 IU) (NHF, Srivastava 2020); AND Individual is using for routine prophylaxis to prevent or reduce the frequency of bleeding episodes; AND Individual has one of the following: A. B. Individual has had one or more episodes of spontaneous bleeding into joint; OR Individual has had one or more episodes of severe, life-threatening, or spontaneous bleeding as determined by the prescriber; OR C. Severe phenotype hemophilia determined by the individual’s risk factors that increase the risk of a clinically significant bleed, including but not limited to, participation in activities likely to cause injury/trauma, procoagulant and anticoagulant protein levels, comorbid conditions affecting functional ability and physical coordination, or history of a clinically significant bleed. Initial requests for Alphanate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) may be approved if the following criteria are met: I. II. Individual has a diagnosis with acquired Factor VIII deficiency; AND Individual is using for the control and prevention of bleeding episodes. Initial requests for Alphanate, Humate-P, Wilate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) may be approved if the following criteria are met: I. II. III. Individual has a diagnosis of severe von Willebrand disease; OR Individual has a diagnosis of mild to moderate von Willebrand disease and use of desmopressin is known or suspected to be inadequate; AND Individual is using for one of the following: A. The treatment of spontaneous or trauma-induced bleeding episodes; OR B. Peri-procedural management for surgical, invasive or interventional radiology procedures. Continuation requests for Alphanate, Humate-P, or Wilate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) may be approved if the following criteria are met: I. Individual has had a positive therapeutic response to treatment (for example, reduction in frequency and/or severity of bleeding episodes). Alphanate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) may not be approved for the following: I. II. Individual has a diagnosis for severe (type 3) von Willebrand Disease; AND Individual is undergoing major surgery; OR III. Individual is using for prophylaxis of spontaneous bleeding episodes in von Willebrand disease. Humate-P and Wilate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) may not be approved for the following: I. Individual is using for prophylaxis of spontaneous bleeding episodes in von Willebrand disease. Alphanate, Humate-P, Wilate (Anti-hemophilic Factor VIII/von Willebrand Factor Complex, Human) may not be approved when the above criteria are not met and for all other indications. Vonvendi (Recombinant von Willebrand Factor Complex) Initial requests for Vonvendi (Recombinant von Willebrand Factor Complex) may be approved if the following criteria are met: I. II. AND III. Individual is 18 years of age or older; AND Individual is using for one of the following: A. B. Individual has a diagnosis of severe von Willebrand disease; OR Individual has a diagnosis of mild to moderate von Willebrand disease and use of desmopressin is known or suspected to be inadequate; Individual is using for one of the following: 6 A. B. Individual is using to treat spontaneous or trauma-induced bleeding episodes, or for peri-procedural management for surgical, invasive or interventional radiology procedures; OR Individual is using as routine prophylaxis to prevent or reduce the frequency of bleeding episodes receiving on-demand therapy. Continuation requests for Vonvendi (Recombinant von Willebrand Factor Complex) may be approved if the following criteria are met: I. Individual has had a positive therapeutic response to treatment (for example, reduction in frequency and/or severity of bleeding episodes). Vonvendi (Recombinant von Willebrand Factor Complex) may not be approved when the above criteria are not met and for all other indications. Coding The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member. Anti-hemophilic Factor VIII, Human plasma-derived (Hemofil M, Koate-DVI,) HCPCS J7190 Factor VIII Anti-hemophilic factor, human, per IU [Hemofil M, Koate DVI,] ICD-10 Diagnosis D66 Hereditary factor VIII deficiency [hemophilia A] D68.00-D68.09 Von Willebrand's disease D68.311 D68.318 D68.4 Z29.8 Acquired hemophilia Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors Acquired coagulation factor deficiency Encounter for other specified prophylactic measure Z79.899 Other long term (current) drug therapy [prophylactic] Anti-hemophilic Factor VIII, Recombinant (Advate, Afstyla, Kogenate FS, Kovaltry, Novoeight, Nuwiq, Recombinate, Xyntha/Xyntha Solofuse) HCPCS J7182 J7185 J7192 J7209 J7210 J7211 Injection, factor VIII, (Anti-hemophilic factor, recombinant), (Novoeight), per IU Injection, factor VIII (Anti-hemophilic factor, recombinant) (Xyntha) (Xyntha Solofuse), per IU Factor VIII (Anti-hemophilic factor, recombinant) per IU, not otherwise specified [Advate, Kogenate-FS, Recombinate] Injection, factor VIII, (Anti-hemophilic factor, recombinant), (Nuwiq), 1 I.U. Injection, factor VIII, (Anti-hemophilic factor, recombinant), (Afstyla), 1 I.U. Injection, factor VIII, (Anti-hemophilic factor, recombinant), (Kovaltry), 1 I.U. ICD-10 Diagnosis D66 Hereditary factor VIII deficiency [hemophilia A] D68.00-D68.09 Von Willebrand's disease D68.311 D68.318 D68.4 Z29.8 Acquired hemophilia Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors Acquired coagulation factor deficiency Encounter for other specified prophylactic measure 7 Z79.899 Other long term (current) drug therapy [prophylactic] Anti-hemophilic Factor VIII, Long Acting Recombinant, pegylated (Adynovate); Jivi (damoctocog alfa pego); Recombinant Anti-hemophilic Factor, Fc Fusion Protein (Elocatate), Factor VIII Recombinant, glycopegylated (Esperoct), glycopegylated (Esperoct), Factor VIII Recombinant Antihemophilic Factor, Fc-VWF-XTEN Fusion Protein (Altuviiio) HCPCS J7205 J7207 J7208 J7204 J7214 Injection, factor VIII Fc fusion protein, (recombinant), per IU [Eloctate] Injection, factor VIII, (anti-hemophilic factor, recombinant), pegylated, 1 I.U. [Adynovate] Injection, factor viii, (antihemophilic factor, recombinant), pegylated-aucl, [Jivi], 1 i.u. Injection, factor viii, (antihemophilic factor (recombinant), glycopegylated-exei, per iu [Esperoct] Injection, Factor VIII/von Willebrand factor complex, recombinant (Altuviiio), per Factor VIII IU ICD-10 Diagnosis D66 Hereditary factor VIII deficiency D68.00-D68.09 Von Willebrand's disease D68.311 Z29.8 Z79.899 Acquired hemophilia Encounter for other specified prophylactic measure Other long term (current) drug therapy [prophylactic] All diagnoses pend for Altuviiio Anti-hemophilic Factor VIII Recombinant, Porcine Sequence (Obizur) HCPCS J7188 J7191 Injection, factor VIII (Anti-hemophilic factor, recombinant), (Obizur), per I.U. Factor VIII, Anti-hemophilic factor (porcine), per IU ICD-10 Diagnosis D68.311 Acquired hemophilia D68.318 D68.4 Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors Acquired coagulation factor deficiency Anti-hemophilic Factor VIII/Von Willebrand Factor Complex (Alphanate, Humate-P, Wilate) HCPCS J7183 J7186 J7187 Injection, Von Willebrand factor complex (human) 1 IU VWF:RCO [Wilate] Injection, antihemophilic factor VIII/Von Willebrand factor complex (human), per factor VIII I.U. [Alphanate] Injection, Von Willebrand factor complex, per IU, VWF:RCO [Humate-P] ICD-10 Diagnosis D66 Hereditary factor VIII deficiency D68.00-D68.09 Von Willebrand's disease D68.311 D68.318 D68.4 Z29.8 Z79.899 Acquired hemophilia Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors Acquired coagulation factor deficiency Encounter for other specified prophylactic measure Other long term (current) drug therapy [prophylactic] Von Willebrand factor, Recombinant (Vonvendi) 8 HCPCS J7179 ICD-10 Diagnosis D68.00-D68.09 D68.311 Z29.8 Injection, Von Willebrand factor (recombinant), (Vonvendi), 1 I.U. VWF:RCO Von Willebrand’s disease Acquired hemophilia Encounter for other specified prophylactic measure Hemlibra (emicizumab) - Anti-hemophilic bispecific factor --- Factor IXa and Factor X HCPCS J7170 ICD-10 Diagnosis D66 Injection, emicizumab-kxwh, 0.5 mg [Hemlibra] [Note: code effective 01/01/2019] Hereditary factor VIII deficiency [hemophilia A] D68.00-D68.09 Von Willebrand's disease D68.311 Z29.8 Z79.899 Acquired hemophilia Encounter for other specified prophylactic measure Other long term (current) drug therapy [prophylactic] Document History Revised: 11/17/2023