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Horizon BCBSNJ
Uniform Medical Policy Manual	Section:	Drugs
	Policy Number:	174
	Effective Date:	07/13/2020

	Original Policy Date:	03/27/2018
	Last Review Date:	06/09/2020
	Date Published to Web:	05/15/2019

Subject:
Hemophilia Products - Factor VIII (Hemophilia A) and Factor IX (Hemophilia B) with inhibitors, Acquired Hemophilia, Congenital Factor VII Deficiency, and Glanzmann’s Thrombasthenia: Novoseven RT and Sevenfact

Description:
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IMPORTANT NOTE:

The purpose of this policy is to provide general information applicable to the administration of health benefits that Horizon Blue Cross Blue Shield of New Jersey and Horizon Healthcare of New Jersey, Inc. (collectively “Horizon BCBSNJ”) insures or administers. If the member’s contract benefits differ from the medical policy, the contract prevails. Although a service, supply or procedure may be medically necessary, it may be subject to limitations and/or exclusions under a member’s benefit plan. If a service, supply or procedure is not covered and the member proceeds to obtain the service, supply or procedure, the member may be responsible for the cost. Decisions regarding treatment and treatment plans are the responsibility of the physician. This policy is not intended to direct the course of clinical care a physician provides to a member, and it does not replace a physician’s independent professional clinical judgment or duty to exercise special knowledge and skill in the treatment of Horizon BCBSNJ members. Horizon BCBSNJ is not responsible for, does not provide, and does not hold itself out as a provider of medical care. The physician remains responsible for the quality and type of health care services provided to a Horizon BCBSNJ member.

Horizon BCBSNJ medical policies do not constitute medical advice, authorization, certification, approval, explanation of benefits, offer of coverage, contract or guarantee of payment.
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Hemophilia is a rare disorder in which the blood does not clot normally. It is a medical condition in which the ability of the blood to clot is severely reduced, causing the sufferer to bleed severely from even a slight injury. The condition is typically caused by a hereditary lack of a coagulation factor, most often factor VIII. Hemophilia A and B are X-linked recessive diseases that present in male children of carrier females. However, hemophilia must sometimes be differentiated from other bleeding disorders when the family history is negative or unknown. Differentiation between hemophilia and other conditions, such as some types of von Willebrand disease or acquired factor inhibitors, and distinction between hemophilia A and B are crucial for appropriate management.

Hemophilia typically refers to an inherited bleeding disorder caused by deficiency of coagulation factor VIII (hemophilia A), factor IX (hemophilia B), or factor XI (hemophilia C). Hemophilia A – Inherited deficiency of factor VIII; is an X-linked recessive disorder. Hemophilia B – Inherited deficiency of factor IX; also called Christmas disease; is an X-linked recessive disorder. Hemophilia A is more common than hemophilia B, representing 80-85% of the total hemophilia population and tends to be more severe. Hemophilia A can also be an acquired which is a bleeding disorder that is caused by autoantibodies that affect clotting factor VIII activity. The main reason for production of factor VIII autoantibodies in a particular individual are not clear, but may involve the presence of certain gene polymorphisms (eg, HLA, CTLA4) and/or autoreactive CD4+ T lymphocytes. It occurs in approximately 1 in 30,000 live male births – approximately half have severe disease (i.e, factor IX activity <1% of normal).

Clinical manifestations of hemophilia relate to bleeding from impaired hemostasis, sequelae from bleeding, or complications of coagulation factor infusion. Patients with moderate hemophilia often bleed in response to minor intercurrent injury and invasive procedures. Bleeding is less frequent than in severe hemophilia and typically occurs four to six times yearly. However, more frequent bleeding may occur if a target joint develops. Some patients with moderate hemophilia may express a more severe phenotype requiring the use of prophylactic treatment regimens. In contrast, individuals with mild hemophilia generally only have bleeding in response to injury/trauma or surgery, and bleeding may not become clinically apparent until later in life. Delayed bleeding can occur after minor surgical procedures such as tooth extraction, even in patients with mild disease.

The diagnostic evaluation in cases of suspected hemophilia typically begins with a thorough review of the patient's personal bleeding history and family history. Screening tests are then performed and the diagnosis is confirmed with a specific clotting factor activity measurement(s) and/or genetic testing. Laboratory testing is similar for the majority of patients with hemophilia. Initial testing includes screening tests of hemostasis, including prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet count. Mixing studies for the aPTT assay are performed if the aPTT is prolonged. If mixing studies show correction, consistent with a factor deficiency rather than an inhibitor, factor activity levels are then measured. An exception is diagnosis in a male neonate with a positive family history, in which factor levels are often measured directly on cord blood.

Table 1: The 2^nd edition World Federation of Hemophilia (WFH) guidelines for management of hemophilia screening tests for hemophilia
Interpretation of screening tests

Possible diagnosis PT (prothrombin time) APTT* (activated partial thromboplastin time) BT (bleeding time) Platelet Count

Normal Normal Normal Normal Normal

Hemophilia A or B** Normal Prolonged* Normal Normal

Platelet defect Normal Normal Normal or prolonged Normal or reduced

*Results of APTT measurements are highly dependent on the laboratory method used for analysis
**The same pattern can occur in presence of FXI, FXII, prekallikrein, or high molecular weight kininogen deficiencies

Table 2: The 2^nd edition World Federation of Hemophilia (WFH) guidelines for management of hemophilia severity classification criteria for hemophilia
Relationship of bleeding severity to clotting factor level

Severity Clotting Factor Level Bleeding Episodes

Severe <1 IU/dl (<0.01 IU/ml) or <1% of normal Spontaneous bleeding into joints or muscles, predominantly in the absence of identifiable hemostatic challenge

Moderate 1-5 IU/dl (0.01-0.05 IU/ml) or 1-5% of normal Occasional spontaneous bleeding; prolongs bleeding with minor trauma or surgery

Mild 5-40 IU/dl (0.05-0.40 IU/ml) or 5-<40% of normal Severe bleeding with major trauma or surgery. Spontaneous bleeding is rare.

Inhibitors are a serious medical problem that can occur when a person with hemophilia has an immune response to treatment with clotting factor concentrates. In the case of an inhibitor, a person's immune system reacts to proteins in factor concentrates as if they were harmful foreign substances. When this happens, antibodies form in the blood to fight against the foreign factor proteins which stops the factor concentrates from being able to fix the bleeding problem. Bleeding is very hard to control in someone with hemophilia who develops inhibitors. In patients with persistent inhibitors, if bleeding into the muscles and joints (the most common type of bleeding in hemophilia) is not controlled, permanent joint damage is likely.

It is possible to get rid of inhibitors using a technique called immune tolerance induction (ITI). With ITI therapy, factor concentrate is given regularly over a period of time until the body is trained to recognize the treatment product without reacting to it. When successful, the inhibitors disappear and the patient’s response to factor concentrates returns to normal. The majority of people who undergo ITI therapy will see an improvement within 12 months, but more difficult cases can take two years or longer.

The amount of inhibitor in an individual is referred to as a titer and it is expressed in Bethesda units. The higher the number of Bethesda units, the more inhibitor that is present. An inhibitor can also be classified as low-responding or high-responding depending on how an individual’s immune system is stimulated based on repeated exposure to factor VIII. If the inhibitor titer is very low (i.e. < 5 Bethesda units) and low-responding, then bleeding episodes in affected individuals can often be treated with replacement factor VIII in higher doses. However, replacement factor VIII is not effective in individuals with a high inhibitor titer (i.e. > 5 Bethesda units). In individuals with higher titer levels, bypassing agents (concentrates of factors that bypass the factor deficiency) are often used. In individuals with higher titer levels, bypassing agents (concentrates of factors that bypass the factor deficiency) are often used to control bleeding episodes. The bypassing agents that are presently available are recombinant activated factor VII (rFVIIa or NovoSeven® RT) or activated prothrombin complex concentrate (aPCC or FEIBA®).

Acquired hemophilia (AH) is a rare autoimmune disorder characterized by bleeding that occurs in patients with a personal and family history negative for hemorrhages. In AH, the body produces inhibitors that attack clotting factors, most often factor VIII. Consequently, affected individuals develop complications associated with abnormal, uncontrolled bleeding into the muscles, skin and soft tissue and during surgery or following trauma. In approximately 50% of patients, there is an identifiable underlying clinical condition; in the other 50% no cause is known. AH should be suspected by the clinical picture and confirmed by an abnormal coagulation test. A diagnosis should be considered in patients with a recent onset of abnormal bleeding and an isolated prolongation of aPTT, especially the elderly and peri- and post-partum women.

Factor VII deficiency is a rare genetic bleeding disorder characterized by a deficiency or reduced activity of clotting factor VII which is caused by mutations of the F7 gene. It is inherited as an autosomal recessive disorder. The symptoms and severity of factor VII deficiency are highly variable; no consistent correlation between the amount of factor VII in the blood and overall severity is seen. Some individuals may not develop any symptoms, including individuals with relatively low levels of factor VII. Other individuals may have mild cases that are only apparent after trauma or surgery. Mild symptoms can include chronic nosebleeds, easy bruising, and bleeding from the gums. Affected women may develop heavy and prolonged periods (menorrhagia). More serious bleeding complications occur in some individuals and may mimic bleeding patterns seen in hemophilia. Bleeding into the joints (hemarthrosis) can result in progressive joint damage and degeneration, eventually limiting the range of motion of an affected joint. Bleeding in the stomach, intestines and urogenital tract can also occur resulting in hematuria or hematochezia.

A diagnosis of factor VII deficiency is based upon identification of characteristic symptoms, a detailed patient history, a thorough clinical evaluation and a variety of specialized blood tests. Tests will include screening coagulation tests that measure how long it takes the blood to clot, specifically aPTT and PT. Individuals with factor VII deficiency have a normal aPTT and a prolonged PT. In affected individuals a factor VII assay will demonstrate reduced activity of factor VII.

Glanzmann thrombasthenia (GT) is a rare inherited blood clotting disorder characterized by the impaired function of platelets. Symptoms of this disorder usually include abnormal bleeding, which may be severe. Prolonged untreated or unsuccessfully treated hemorrhaging associated with Glanzmann thrombasthenia may be life threatening. Most individuals affected with Glanzmann thrombasthenia have a normal number of platelets but have a prolonged bleeding time, which means it takes longer than usual for a standardized cut to stop bleeding. Platelet aggregation studies are abnormal and show that platelets are not able to clump together when stimulated as they should to form platelet aggregates. Glanzmann thrombasthenia is definitively diagnosed by tests that determine if there is a deficiency of the aIIbb3 (GPIIb/GPIIIa) receptor. These tests usually involve monoclonal antibodies and flow cytometry. Genetic tests can identify the DNA mutations responsible for the disorder.

NovoSeven® RT is a recombinant factor VIIa product manufactured by Novo Nordisk. It does not contain human blood or plasma and consequently, there is no risk of blood-borne viruses or other such pathogens. NovoSeven has been well-tolerated and associated with few side effects. Risk of thrombotic adverse effects is below 1% for individuals with AH. NovoSeven has been approved by the FDA for use as a bypassing agent for the treatment of individuals with acquired hemophilia. In 2014 it was approved to treat Glanzmann thrombasthenia. This medication is indicated to treat bleeding episodes and perioperative management when platelet transfusions, with or without antibodies to platelets, are not effective.

In April 2020, the U.S. Food & Drug Administration (FDA) approved Sevenfact [coagulation factor VIIa (recombinant)-jncw] which is a coagulation factor VIIa concentrate indicated for the treatment and control of bleeding episodes occurring in adults and adolescents (12 years of age and older) with hemophilia A or B with inhibitors. The approval of Sevenfact was based on data from the pivotal phase 3 trial, PERSEPT 1. This clinical trial evaluated 468 mild, moderate, and severe bleeding episodes in 27 adolescent and adult hemophilia A and B patients with inhibitors in a randomized, multicenter, open-label, two initial dose regimens, cross-over trial design. Both initial dose regimens met the primary endpoint of bleed control by 12 hours: 91% of mild or moderate bleeding episodes treated with an initial 225 mcg/kg dose achieved hemostatic efficacy at 12 hours; the median time to hemostatic response was 3 hours, corresponding to 1 dose of Sevenfact. The median time to hemostatic response for bleeding episodes treated with an initial 75 mcg/kg dose was 6 hours (median of 2 doses Sevenfact), with 82% of bleeding episodes achieving hemostatic efficacy at 12 hours. By 24 hours, hemostatic efficacy was retained in 97.6% of bleeding episodes treated with the 75 mcg/kg dose regimen, and 99.5% of bleeding episodes treated with the 225 mcg/kg dose regimen, without requiring any alternative therapy.

Policy:
(NOTE: For Medicare Advantage, please refer to the Medicare Coverage Section below for coverage guidance.)

I. When anti-hemophilia products are considered medically necessary, initial therapy will be approved based on the following:

i. (i.e., severe = <1% factor activity, moderate = ≥1 to ≤5% factor activity, mild = >5 to 40% factory activity)

II. Novoseven RT is considered medically necessary for any of the following FDA-approved conditions:

AND

III. Sevenfact is considered medically necessary for any of the following FDA-approved conditions:

AND

IV. When medically necessary, Novoseven RT will be approved based on the following FDA approved dosing (see table below):

· The cumulative amount of medication(s) the patient has on-hand will be taken into account when authorizing. The authorization will allow up to 5 doses on-hand for the treatment of acute bleeding episodes as needed for the duration of the authorization.

i. Unless otherwise specified, the authorization is valid for 1 month

· Factor dose should not exceed +1% of the prescribed dose and a maximum of three vials may be dispensed per dose. If unable to provide factor dosing within required range of prescribed dose, then rendering provider must provide proof of all available vial sizes from the manufacturer prior to dispensing factor product and the lowest possible dose able to be achieved above +1% should be dispensed. Prescribed dose should not be increased to meet assay management requirements.

Novoseven RT

Indication	Dose
Control and prevention of bleeding Congenital Hemophilia A or B with inhibitors	Minor 90 mcg/kg every 2 hours, adjustable based on severity of bleeding until hemostasis is achieved. Major 90 mcg/kg every 3-6 hours after hemostasis is achieved for severe bleeds
Control and prevention of bleeding episodes Acquired Hemophilia	70-90 mcg/kg every 2-3 hours until hemostasis is achieved
Control and prevention of bleeding episodes Congenital Factor VII deficiency	15-30 mcg/kg every 4-6 hours until hemostasis is achieved
Control and prevention of bleeding episodes Glanzmann’s Thrombasthenia	90 mcg/kg every 2-6 hours until hemostasis is achieved
Perioperative management Congenital Hemophilia A or B with inhibitors	Minor 90 mcg/kg immediately before surgery, repeat every 2 hours during surgery. 90 mcg/kg every 2 hours after surgery for 48 hours, then every 2-6 hours until healing has occurred. Major 90 mcg/kg immediately before surgery, repeat every 2 hours during surgery. 90 mcg/kg every 2 hours after surgery for 5 days, then every 4 hours until healing has occurred.
Perioperative management Acquired Hemophilia	70-90 mcg/kg immediately before surgery and every 2-3 hours for the duration of surgery and until hemostasis is achieved
Perioperative management Congenital Factor VII deficiency	15-30 mcg/kg immediately before surgery and every 4-6 hours for the duration of surgery and until hemostasis is achieved
Perioperative management Glanzmann’s Thrombasthenia	90 mcg/kg immediately before surgery and repeat every 2 hours for the duration of the procedure. 90 mcg/kg every 2-6 hours to prevent post-operative bleeding

V. When medically necessary, Sevenfact will be approved based on the following FDA approved dosing (see table below):

Sevenfact

Indication

Dose

Control and treatment of bleeding: Congenital Hemophilia A or B with inhibitors

Mild or Moderate
75 mcg/kg every 3 hours until hemostasis is achieved
or
Initial dose of 225 mcg/kg. If hemostasis is not achieved within 9 hours, additional 75 mcg/kg doses may be administered every 3 hours as needed to achieve homeostasis

*Consider alterative treatments if successful control of bleeding does not occur within 24 hours of the first administration of Sevenfact

Severe
225 mcg/kg initially, followed if necessary 6 hours later with 75 mcg/kg every 2 hours until hemostasis is achieved

VI. For continuing therapy, Novoseven RT is considered medically necessary and approved when ALL of the following criteria are met:

AND

o Patients with disseminated intravascular coagulation (DIC), advanced atherosclerotic disease, crush injury, septicemia, or concomitant treatment with aPCCs/PCCs (activated or nonactivated prothrombin complex concentrates) and uncontrolled post-partum hemorrhage have increased risk of developing thromboembolic events due to circulating tissue factor (TF) or predisposing coagulopathy. Exercise caution when administering Novoseven RT to patients with an increased risk of thromboembolic complications. Monitor patients who receive Novoseven RT for development of signs or symptoms of activation of the coagulation system or thrombosis.

AND

o Factor VII deficient patients should be monitored for prothrombin time (PT) and factor VII coagulant activity before and after administration of Novoseven RT. If factor VIIa activity fails to reach the expected level, or prothrombin time is not corrected, or bleeding is not controlled after treatment with the recommended doses, antibody formation may be suspected and analysis for antibodies should be performed.

VII. For continuing therapy, Sevenfact is considered medically necessary and approved when ALL of the following criteria are met:

AND

o Patients with concomitant treatment with aPCCs/PCCs (activated or nonactivated prothrombin complex concentrates) or other hemostatic agents, history of atheroslcerotic disease, coronray artery disease, cerebrovascular disease, crush injury, septicemia, or thromboembolism have an increased risk of developing thromboembolic events . Monitor patients who receive Sevenfact for development of signs or symptoms of activation of the coagulation system or thrombosis.

AND

o If treatment with Sevenfact does not result in adequate hemostasis, then suspect development of neutralizing antibody as the possible cause and perform testing as clinically indicated. Neutralizing antibodies to other Factor VIIa-containing products have been observed in congenital Factor VII-deficient patients. Sevenfact has not been studied in this patient population.

VIII. Novoseven RT is considered medically necessary for the following off-label uses:
A. Hemophilia A (congenital factor VIII deficiency) with acquired inhibitors to Factor VIII

Routine prophylaxis to prevent or reduce the frequency of bleeding episodes when the following criteria are also met:
- Patient has at least 2 documented episodes of spontaneous bleeding into joints; OR
- Patient has documented trial and failure of Immune Tolerance Induction (ITI); AND
- Patient has documented trial and failure of or is contraindicated to Hemlibra

B. Hemophilia B (congenital factor IX deficiency) with acquired inhibitors to Factor IX

Routine prophylaxis to prevent or reduce the frequency of bleeding episodes when the following criteria are also met:
· Patient has at least 2 documented episodes of spontaneous bleeding into joints; OR
· Patient has documented trial and failure of Immune Tolerance Induction (ITI)

IX. All other uses of Novoseven RT and Sevenfact are considered investigational.

Medicare Coverage:
Hemophilia Products - Factor VIII (Hemophilia A) and Factor IX (Hemophilia B) with inhibitors, Acquired Hemophilia, Congenital Factor VII deficiency, and Glanzmann’s Thrombasthenia: Novoseven RT are covered when NCD 110.3 and LCD L35111 criteria is met. For additional information and eligibility, refer to NCD 110.3 and L35111.
National Coverage Determination (NCD) for Anti-Inhibitor Coagulant Complex (AICC) (110.3). Available to be accessed at CMS National Coverage Determinations (NCDs) Alphabetical Index search page: https://www.cms.gov/medicare-coverage-database/indexes/ncd-alphabetical-index.aspx.

Local Coverage Determination (LCD): Hemophilia Factor Products (L35111). Available to be accessed at Novitas Solutions, Inc., Medical Policy Search page: https://www.novitas-solutions.com/webcenter/portal/MedicareJL/LcdSearch?_afrLoop=90769712476969#!%40%40%3F_afrLoop%3D90769712476969%26centerWidth%3D100%2525%26leftWidth%3D0%2525%26rightWidth%3D0%2525%26showFooter%3Dfalse%26showHeader%3Dfalse%26_adf.ctrl-state%3D63y7eftob_46.

Medicaid Coverage:
For Horizon NJ Health members, please follow this link for the corresponding HNJH drug policy https://services3.horizon-bcbsnj.com/ddn/NJhealthWeb.nsf

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Horizon BCBSNJ Medical Policy Development Process:

This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.

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Index:
Hemophilia Products - Factor VIII (Hemophilia A) and Factor IX (Hemophilia B) with inhibitors, Acquired Hemophilia, Congenital Factor VII Deficiency, and Glanzmann’s Thrombasthenia: Novoseven RT
Novoseven RT
Factor VIII and Factor IX with Inhibitors
Hemophilia A and Hemophilia B with Inhibitors
Acquired Hemophilia
Congenital Factor VII Deficiency
Glanzmann’s Thrombasthenia
Sevenfact

References:
1. NovoSeven RT [package insert]. Bagsvaerd, Denmark; Novo Nordisk; January 2019.

2. MASAC RECOMMENDATIONS CONCERNING PRODUCTS LICENSED FOR THE TREATMENT OF HEMOPHILIA AND OTHER BLEEDING DISORDERS. 2013 National Hemophilia Foundation. MASAC Document #240; February 2016. Available at: http://www.hemophilia.org. Accessed June 2016.

3. First Coast Service Options, Inc. Local Coverage Determination (LCD): Hemophilia Clotting Factors (L33684). Centers for Medicare & Medicaid Services, Inc. Updated on 01/21/2016 with effective date 01/01/2016. Accessed February 2016.

4. Novitas Solutions, Inc. Local Coverage Determination (LCD): Hemophilia Clotting Factors (L35111). Centers for Medicare & Medicaid Services, Inc. Updated on 01/22/2016 with effective date 01/01/2016. Accessed February 2016.

5. Annual Review of Factor Replacement Products. Oklahoma Health Care Authority Review Board. Updated April 2016. Access June 2016.

6. Graham A1, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia. 2014 Mar;20(2):226-9.

7. Mingot-Castellano, et al. Application of Pharmacokinetics Programs in Optimization of Haemostatic Treatment in Severe Hemophilia a Patients: Changes in Consumption, Clinical Outcomes and Quality of Life. Blood. 2014 December; 124 (21).

8. Blanchette VS, Key NS, Ljung LR, et al. Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost 2014; 12:1935.

9. Guidelines for the management of hemophilia, 2^nd edition. 2012 World Federation of Hemophilia. July 2012. Available at: https://www1.wfh.org/publication/files/pdf-1472.pdf. Accessed March 2018.

10. Collins PW, Chalmers E, Hart DP, et al. UK Haemophilia Centre Doctors. Diagnosis and treatment of factor VIII and IX inhibitors in congenital haemophilia: (4^th edition). UK Haemophilia Centre Doctors Organization. Br J Haematol. 2013 Jan; 160(2): 153-70. Available on PubMed.

11. World Federation of Hemophilia.Glanzmann thrombasthenia. Montreal, Quebec. Updated May 2012.. https://www.wfh.org/en/page.aspx?pid=658. Accessed March 8, 2018.

12. Hartung H. Factor VII Deficiency. National Organization for Rare Disorders. Danbury, CT. Updated 2016. Available at: https://rarediseases.org/rare-diseases/factor-vii-deficiency/. Accessed on March 2018.

13 Coller BS, Rockerfeller D. Glanzmann Thrombasthenia. National Organization for Rare Disorders. Danbury, CT. Updated 2015. Available at: https://rarediseases.org/rare-diseases/glanzmann-thrombasthenia/. Accessed on March 2018.

14. Baudo F, Niguarda O. Acquired Hemophilia. National Organization for Rare Disorders. Danbury, CT. Updated 2016. Available at: https://rarediseases.org/rare-diseases/acquired-hemophilia/. Accessed on March 2018.

15. MASAC recommendation regarding prophylaxis with bypassing agents in patients with hemophilia and high titer inhibitors. National Hemophilia Foundation. Updated October 6, 2013. New York, NY. https://www.hemophilia.org/sites/default/files/document/files/masac220.pdf. Accessed on August 3, 2018.

16. Sevenfact [package insert]. Les Ulis, France; LFB S.A., April 2020.

Codes:
(The list of codes is not intended to be all-inclusive and is included below for informational purposes only. Inclusion or exclusion of a procedure, diagnosis, drug or device code(s) does not constitute or imply authorization, certification, approval, offer of coverage or guarantee of payment.)

CPT*

HCPCS

J7189

* CPT only copyright 2020 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

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Medical policies can be highly technical and are designed for use by the Horizon BCBSNJ professional staff in making coverage determinations. Members referring to this policy should discuss it with their treating physician, and should refer to their specific benefit plan for the terms, conditions, limitations and exclusions of their coverage.

The Horizon BCBSNJ Medical Policy Manual is proprietary. It is to be used only as authorized by Horizon BCBSNJ and its affiliates. The contents of this Medical Policy are not to be copied, reproduced or circulated to other parties without the express written consent of Horizon BCBSNJ. The contents of this Medical Policy may be updated or changed without notice, unless otherwise required by law and/or regulation. However, benefit determinations are made in the context of medical policies existing at the time of the decision and are not subject to later revision as the result of a change in medical policy
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