Subject: Novoseven RT 1947-A SGM P2022

Drug

NOVOSEVEN RT  (coagulation factor VIIa [recombinant]))

Policy:

I.    INDICATIONS

The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy. 

A.   FDA-Approved Indications
      1.   Hemophilia A or hemophilia B with inhibitors
      2.   Congenital factor VII deficiency
      3.   Glanzmann’s thrombasthenia
      4.   Acquired hemophilia 

B.   Compendial Uses
      1.   Acquired von Willebrand syndrome
      2.   Inhibitors to factor XI 

All other indications are considered experimental/investigational and not medically necessary. 

II.  PRESCRIBER SPECIALTIES 

Must be prescribed by or in consultation with a hematologist. 

III. CRITERIA FOR INITIAL APPROVAL 

A.   Congenital Factor VII Deficiency
       Authorization of 12 months may be granted for treatment of congenital factor VII deficiency. 

B.   Hemophilia A with Inhibitors
      Authorization of 12 months may be granted for treatment of hemophilia A with inhibitors (see Appendix)
      when the inhibitor titer is ≥ 5 Bethesda units per milliliter (BU/mL) or the member has a history of an
      inhibitor titer ≥ 5 BU. 

C.   Hemophilia B with Inhibitors
      Authorization of 12 months may be granted for treatment of hemophilia B with inhibitors (see Appendix)
      when the inhibitor titer is ≥ 5 Bethesda units per milliliter (BU/mL) or the member has a history of an inhibitor
      titer ≥ 5 BU.

D.   Glanzmann’s Thrombasthenia
       Authorization of 12 months may be granted for treatment of Glanzmann’s thrombasthenia.

E.   Acquired Hemophilia
      Authorization of 12 months may be granted for treatment of acquired hemophilia. 

F.   Acquired von Willebrand Syndrome
      Authorization of 12 months may be granted for treatment of acquired von Willebrand syndrome when
      other therapies failed to control the member’s condition (e.g., desmopressin or factor VIII/von Willebrand factor). 

G.   Inhibitors to Factor XI
      Authorization of 12 months may be granted for treatment of inhibitors to factor XI.

IV.  CONTINUATION OF THERAPY 

Authorization of 12 months may be granted for continued treatment in members requesting reauthorization for an indication listed in Section III when the member is experiencing benefit from therapy (e.g., reduced frequency or severity of bleeds).

Special Notes:

V.   APPENDIX 

Appendix: Inhibitors - Bethesda Units (BU)
The presence of inhibitors is confirmed by a specific blood test called the Bethesda inhibitor assay. 

• High-titer inhibitors:
  • > 5 BU/mL
  • Inhibitors act strongly and quickly neutralize factor 

• Low-titer inhibitors:
  • < 5 BU/mL
  • Inhibitors act weakly and slowly neutralize factor

Place of Service:

Outpatient

The above policy is based on the following references:

1. NovoSeven RT [package insert]. Plainsboro, NJ: Novo Nordisk Inc.; July 2020.
2. National Institutes of Health. The diagnosis, evaluation, and management of von Willebrand disease. Bethesda, MD: US Dept of Health and Human Services, National Institutes of Health; 2007. NIH publication No. 08-5832
3. Tiede A, Rand J, Budde U, et al. How I treat the acquired von Willebrand syndrome. Blood. 2011;117(25):6777-85.
4. Federici AB, Budde U, Castaman G, Rand JH, Tiede A. Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. Semin Thromb Hemost. 2013;39(2):191-201.
5. O’Connell NM. Factor XI deficiency – from molecular genetics to clinical management. Blood Coagul Fibrinolysis. 2003;14(Suppl 1):S59-S64.
6. Salomon O, Zivelin A, Livnat T, Seligsohn U. Inhibitors to factor XI in patients with severe factor XI deficiency. Semin Hematol. 2006;43(1 Suppl 1):S10-S12.
7. Srivastava A, Santagostino E, Dougall A, et al. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia. 2020;26 Suppl 6:1-158. doi:10.1111/hae.14046.
8. National Hemophilia Foundation. MASAC recommendations concerning products licensed for the treatment of hemophilia and other bleeding disorders. Revised August 2020. MASAC Document #263. https://www.hemophilia.org/sites/default/files/document/files/263_treatment.pdf. Accessed December 7, 2021.
9. World Federation of Hemophilia. What are inherited platelet function disorders? http://www1.wfh.org/publication/files/pdf-1336.pdf. 2010. Accessed December 7, 2021.
10. World Federation of Hemophilia. Platelet function disorders. http://www1.wfh.org/publication/files/pdf-1147.pdf. 2008. Accessed December 7, 2021.
11. Rajpurkar M, Chitlur M, Recht M, Cooper DL. Use of recombinant activated factor VII in patients with Glanzmann’s thrombasthenia: a review of the literature. Haemophilia. 2014;20(4):464-471.
12. Duga S, Salomon O. Congenital factor XI deficiency: an update. Semin Thromb Hemost. 2013;39(6):621-631.

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

May 16, 2022