Coverage Policies

Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Ryplazim (plasminogen, human-tvmh) requires prior authorization.

2)    Ryplazim is indicated for the treatment of patients with plasminogen deficiency type 1 (hypoplasminogenemia).

Initial Approval Criteria A. Plasminogen Deficiency Type 1 (must meet all):

1. Diagnosis of symptomatic congenital plasminogen deficiency (C-PLGD) as evidenced by documentation of two of the following (a - c):

a. Presence of a PLG mutation;

b. Plasminogen activity level ≤ 45%;

c. Signs or symptoms consistent with C-PLGD (see Appendix D);

2. Prescribed by or in consultation with a hematologist;

3. Age ≥ 11 months;

4. Dose does not exceed 6.6 mg/kg every second, third, or fourth day (based upon individual pharmacokinetics).

Approval duration: 6 months

Continued Therapy

A. Plasminogen Deficiency Type 1 (must meet all):

1. Currently receiving medication via Centene benefit or member has previously met initial approval criteria;

2. Member is responding positively to therapy as evidenced by, including but not limited to, improvement in C-PLGD-associated signs or symptoms (e.g., improvement in the size of visible lesions, imaging of nonvisible lesions, or spirometry if pulmonary involvement (see Appendix D));

3. If request is for a dose increase, new dose does not exceed 6.6 mg/kg every second, third, or fourth day (based upon individual pharmacokinetics).

Approval duration: 12 months

Codes Used In This BI:

1)    J2998

1. Ryplazim Prescribing Information. Prometic Bioproductions Inc: Laval, Quebec, Canada; June 2021. Available at: https://www.fda.gov/media/149806/download. Accessed July 6, 2021.

2. Product Pipeline: Plasminogen Deficiency. Liminal BioSciences, Inc. Available at: https://liminalbiosciences.com/pipeline/plasminogen/plasminogen-deficiency-clinical-trials/. Accessed October 1, 2020.

3. Shapiro, Amy D. et al. An international registry of patients with plasminogen deficiency (HISTORY). Haematologica. 2020 Mar; 105(3):554-561.

4. Shapiro, Amy D. et al. Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency. Blood. 2018 Mar 22; 131(12):1301-1310.

5. Mehta R, Shapiro AD. Plasminogen deficiency. Haemophilia. 2008; 14, 1261–1268. DOI: 10.1111/j.1365-2516.2008.01825.x.

6. Schuster V, Hugle B, Tefs K. Plasminogen deficiency. J Thromb Haemost 2007; 5:2315–22.

7. Tefs K, Gueorguieva M, Klammt J, et al. Molecular and clinical spectrum of type I plasminogen deficiency: a series of 50 patients. Blood, 1 November 2006; 108(9):3021-3026.

8. A treatment protocol for expanded access administration of Prometic plasminogen due to closure of clinical trial. Trial record 1 of 2 for: 2002C011G. ClinicalTrials.gov Identifier: NCT03642691. Available at: https://clinicaltrials.gov/ct2/show/NCT03642691?term=2002C011G&draw=2&rank=1. Accessed October 1, 2020.

9. A study of Prometic plasminogen IV infusion in subjects with hypoplasminogenemia. Trial record 2 of 2 for: 2002C011G. ClinicalTrials.gov Identifier: NCT02690714. Available at: https://clinicaltrials.gov/ct2/show/NCT02690714?term=2002C011G&draw=2&rank=2. Accessed October 1, 2020.

10. Type 1 plasminogen deficiency. Genetic and Rare Diseases Information Center. National Institutes of Health. Available at https://rarediseases.info.nih.gov/diseases/4380/type-1-plasminogen-deficiency. Accessed October 6, 2020.