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Drug and Biologic Coverage Policy Effective Date ............................................ 7/1/2021 Next Review Date… ..................................... 7/1/2022 Coverage Policy Number .................................. 8007 Clotting Factors and Antithrombin Table of Contents Related Coverage Resources Coverage Policy ................................................... 1 Caplacizumab-yhdp - 1906 General Background ............................................ 6 Recommended Dosing ...................................... 17 Coding/Billing Information .................................. 36 References ........................................................ 37 INSTRUCTIONS FOR USE The following Coverage Policy applies to health benefit plans administered by Cigna Companies. Certain Cigna Companies and/or lines of business only provide utilization review services to clients and do not make coverage determinations. References to standard benefit plan language and coverage determinations do not apply to those clients. Coverage Policies are intended to provide guidance in interpreting certain standard benefit plans administered by Cigna Companies. Please note, the terms of a customer’s particular benefit plan document [Group Service Agreement, Evidence of Coverage, Certificate of Coverage, Summary Plan Description (SPD) or similar plan document] may differ significantly from the standard benefit plans upon which these Coverage Policies are based. For example, a customer’s benefit plan document may contain a specific exclusion related to a topic addressed in a Coverage Policy. In the event of a conflict, a customer’s benefit plan document always supersedes the information in the Coverage Policies. In the absence of a controlling federal or state coverage mandate, benefits are ultimately determined by the terms of the applicable benefit plan document. Coverage determinations in each specific instance require consideration of 1) the terms of the applicable benefit plan document in effect on the date of service; 2) any applicable laws/regulations; 3) any relevant collateral source materials including Coverage Policies and; 4) the specific facts of the particular situation. Coverage Policies relate exclusively to the administration of health benefit plans. Coverage Policies are not recommendations for treatment and should never be used as treatment guidelines. In certain markets, delegated vendor guidelines may be used to support medical necessity and other coverage determinations. Coverage Policy The Clotting Factors and Antithrombin coverage policy includes the following products: • Antithrombin III ® o Human plasma-derived: Thrombate III ® o Recombinant: ATryn • Anti-Inhibitor Coagulant Complex: FEIBA™ • Coagulation Factor X, (human): Coagadex® • Factor VIIa: NovoSeven® RT, SEVENFACT® • Factor VIII - Antihemophilic factor ® ® o Human plasma-derived: Hemofil M, Koate ® ® ® ® ® ® o Recombinant: Advate , Kogenate FS, Novoeight , Nuwiq , Recombinate , Xyntha ® o Recombinant, Fc fusion protein: Eloctate ® o Recombinant, human DNA sequence derived: Kovaltry ® o Recombinant, glycoPEGylated: Esperoct ™ ® o Recombinant, pegylated: Adynovate , Jivi ™ o Recombinant, porcine sequence: Obizur ® o Recombinant, single chain: Afstyla • Factor VIII-Antihemophilic factor/von Willebrand factor complex (human): Alphanate®, Humate-P® • Factor IX Page 1 of 39 Coverage Policy Number: 8007 ® ® o Human plasma-derived: AlphaNine SD, Mononine ® o Complex (human plasma-derived): Profilnine ® ® ™ o Recombinant: BeneFIX , Ixinity , Rixubis ® o Recombinant, albumin fusion protein: Idelvion ® o Recombinant, glycoPEGylated: Rebinyn ® o Recombinant, Fc fusion protein: Alprolix • Factor XIII ® o Concentrate (human plasma-derived): Corifact ® o Recombinant (Coagulation Factor XIII A-Subunit): Tretten • Fibrinogen concentrate: RiaSTAP®, Fibryga® • von Willebrand factor, recombinant: Vonvendi™ • von Willebrand factor/coagulation factor VIII complex (human plasma-derived): Wilate® NOTE: Each Clotting Factor product has unique indications and uses and are only approved for use as listed in the criteria below. Clotting Factor products are considered medically necessary when the following criteria are met: Product Criteria for Use Advate Individual with factor VIII deficiency (hemophilia A) for ANY of the following: (factor VIII, recombinant) • On-demand treatment and control of bleeding episodes • Peri-operative management of bleeding • Routine prophylaxis to reduce the frequency of bleeding episodes Adynovate Individual with factor VIII deficiency (hemophilia A) for ANY of the following: (factor VIII - • On-demand treatment and control of bleeding episodes antihemophilic factor • Peri-operative management of bleeding [recombinant], • Routine prophylaxis to reduce the frequency of bleeding episodes pegylated) Afstyla Individual with factor VIII deficiency (hemophilia A) for ANY of the following: (antihemophilic factor, • On-demand treatment and control of bleeding episodes recombinant, single • Peri-operative management of bleeding chain) • Routine prophylaxis to reduce the frequency of bleeding episodes Alphanate EITHER of the following: (factor VIII - • Treatment and prevention of bleeding in hemophilia A antihemophilic factor/von • Von Willebrand disease (VWD) if BOTH of the following: Willebrand factor o For use in surgical and/or invasive procedures complex) o When there is a failure/inadequate response, contraindication, intolerance, not a candidate for, or inability to obtain BOTH of the following: . Concentrated intranasal desmopressin (Stimate) . Parenteral desmopressin (DDAVP injection) Alphanate is NOT indicated for severe VWD (type 3) undergoing major surgery. Alphanine SD Individual with factor IX deficiency (hemophilia B) for prevention or control of (factor IX, human bleeding. plasma-derived) Alprolix Individual with factor IX deficiency (hemophilia B) for ANY of the following: (factor IX, recombinant, • On-demand treatment and control of bleeding episodes Fc fusion protein) • Peri-operative management of bleeding • Routine prophylaxis to reduce the frequency of bleeding episodes ATryn Prevention of peri-operative and peri-partum thromboembolic events in hereditary [antithrombin III antithrombin deficient individuals (recombinant)] BeneFIX Individual with factor IX deficiency (hemophilia B) for EITHER of the following: (factor IX, recombinant) • Prevention or control of bleeding Page 2 of 39 Coverage Policy Number: 8007 Product Criteria for Use • Peri-operative management of bleeding Coagadex Individual with coagulation factor X deficiency with ANY of the following: (coagulation Factor X • On-demand treatment and control of bleeding episodes [human]) • Peri-operative management of bleeding in individuals with mild or moderate hereditary factor X deficiency • Routine prophylaxis to reduce the frequency of bleeding episodes Corifact Individual with factor XIII deficiency for EITHER of the following: [factor XIII Concentrate • Routine prophylactic treatment (human plasma-derived)] • Peri-operative management of bleeding Eloctate Individual with factor VIII deficiency (hemophilia A) for ANY of the following: (antihemophilic factor • On-demand treatment and control of bleeding episodes [recombinant, Fc fusion • Peri-operative management of bleeding protein]) • Routine prophylaxis to reduce the frequency of bleeding episodes Esperoct Individual with factor VIII deficiency (hemophilia A) for ANY of the following: (antihemophilic factor • On-demand treatment and control of bleeding episodes [recombinant] • Peri-operative management of bleeding glycoPEGylated-exei) • Routine prophylaxis to reduce the frequency of bleeding episodes FEIBA ONE of the following: (anti-inhibitor coagulant I. Hemophilia A with Inhibitors and BOTH of the following: complex) • Individual meets ONE of the following: o Positive inhibitor titer ≥ 5 Bethesda Units o History of anamnestic response to Factor VIII replacement therapy, which, according to the prescriber, precludes the use of Factor VIII replacement to treat bleeding episodes o History of refractory response to increased Factor VIII dosing, which, according to the prescriber, precludes the use of Factor VIII replacement to treat bleeding episodes • Prescribed by or in consultation with a hematologist II. Hemophilia B with Inhibitors and BOTH of the following: • Individual meets ONE of the following: o Positive inhibitor titer ≥ 5 Bethesda Units o History of anamnestic response to Factor IX replacement therapy, which, according to the prescriber, precludes the use of Factor IX replacement to treat bleeding episodes o History of refractory response to increased Factor IX dosing, which, according to the prescriber, precludes the use of Factor IX replacement to treat bleeding episodes • Prescribed by or in consultation with a hematologist Fibryga Treatment of acute bleeding episodes in an individual with congenital fibrinogen (fibrinogen concentrate) deficiency (afibrinogenemia and hypofibrinogenemia) confirmed by both of the following: • Prolonged activated partial thromboplastin time and prothrombin time at baseline, as defined by the laboratory reference values; AND • Lower than normal plasma functional and antigenic fibrinogen levels at baseline, as defined by the laboratory reference values Hemofil M Individual with factor VIII deficiency (hemophilia A) for ANY of the following: (factor VIII, human • On-demand treatment and control of bleeding episodes plasma-derived) • Peri-operative management of bleeding • Routine prophylaxis
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