RYPLAZIM (plasminogen, human-tvmh)

RYPLAZIM (plasminogen, human-tvmh)

Medical Administration - intravenous

Diagnosis considered for coverage: 

• Indicated for the treatment of patients with plasminogen deficiency type 1 (hypoplasminogenemia).

Coverage Criteria:

For diagnosis of plasminogen deficiency type 1:

• Dose does not exceed 6.6 mg/kg administered intravenously every 2 to 4 days (initiate at a frequency of every three days), AND
• Prescribed by or in consultation with a hematologist, AND
• Chart note documentation is provided showing the diagnosis of plasminogen deficiency type 1 (hypoplasminogenemia) is confirmed by the following:
  • Deficiency of plasminogen activity evidenced by a level of less than or equal to 50%, as confirmed by a chromogenic assay, AND
  • Abnormal plasminogen antigen plasma level of less than 9 mg/dL, as confirmed by an enzyme-linked immunosorbent assay, AND
• Presence of clinical symptoms and signs of the disease (e.g., ligneous conjunctivitis, ligneous gingivitis or gingival overgrowth, occlusive hydrocephalus, vision abnormalities, respiratory distress/obstruction, abnormal wound healing)

Reauthorization Criteria:

For diagnosis of plasminogen deficiency type 1:

• Dose does not exceed 6.6 mg/kg administered intravenously every 2 to 4 days, AND
• Documentation of positive clinical response to therapy (e.g., plasminogen activity trough level increased by at least 10 percentage points from baseline, improvement or resolution of lesions)

Coverage Duration: 

• Initial: 6 months
• Reauthorization: 1 year

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information: 

•    The dosing frequency is individualized based on the patient’s trough plasminogen activity level and lesion resolution (see the Prescribing Information for detailed recommendations).
•    Warnings and precautions include:

• Bleeding
• Tissue Sloughing
• Transmission of infectious agents
• Hypersensitivity reactions
• Neutralizing antibodies
• Laboratory abnormalities

Policy Updates:

• 5/17/2022 – New policy approved by P&T.

References:

• Mehta R and Shapiro AD. Plasminogen deficiency. Haemophilia. 2008; 14:1261-1268. doi: 10.1111/j.1365-2516.2008.01825.x 
• Ryplazim [package insert], Laval, Quebec, Canada: Prometic Bioproduction Inc.; June 2021. 
• Schuster V, Hügle B, Tefs K. Plasminogen deficiency. J Thromb Haemost. 2007;5(12):2315-2322. doi:10.1111/j.1538-7836.2007.02776.x 
• Schuster V, Seregard S. Ligneous conjunctivitis. Surv Ophthalmol. 2003;48(4):369-388. doi:10.1016/s0039-6257(03)00056-0 
• Shapiro AD, Nakar C, Parker JM, et al. Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency. Blood. 2018;131(12):1301-1310. doi:10.1182/blood-2017-09-806729