CurrentUnitedHealthcarePolicy CSOH2025D0070.C

Ryplazim (Plasminogen, Human-Tvmh) (for Ohio Only)

Medical benefit drug policy governing coverage and prior authorization criteria for Ryplazim for members in Ohio; applies to treatment of plasminogen deficiency type 1 and excludes idiopathic pulmonary fibrosis.

Policy Summary

PayerUnitedHealthcare

PolicyRyplazim (Plasminogen, Human-Tvmh) (for Ohio Only)

Policy CodePolicy CSOH2025D0070.C

Change TypeNo material clinical or coverage changes

Effective DateMay 1, 2025

Next Review DateN/A

Key ActionObtain prior authorization with documentation of plasminogen activity ≤45%, clinical signs/symptoms, hematology prescriber or consultation, and dosing per FDA labeling.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

J2998HCPCS for Ryplazim

E88.02ICD-10 code

≤45%plasminogen activity threshold

12 monthsmax initial auth

78% / 75%trial lesion response

Coverage Criteria for Ryplazim (plasminogen, human-tvmh)

Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

Initial therapy for Ryplazim: Diagnosis of hypoplasminogenemia as measured by plasminogen activity level ≤ 45% of laboratory standard; presence of clinical signs and symptoms of the disease (e.g., ligneous conjunctivitis, gingivitis, tonsillitis, abnormal wound healing, etc.); prescribed by or in consultation with a hematologist; dosing is in accordance with the U.S. Food and Drug Administration (FDA) approved labeling; initial authorization will be for no more than 12 months.plasminogen activity ≤ 45%

From Coverage Rationale

Continuation Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

Continuation therapy for Ryplazim: Patient has previously received treatment with Ryplazim; patient has experienced a positive clinical response to Ryplazim therapy (e.g., reduction in lesion number/size, improved wound healing, or plasminogen activity trough level increased by at least 10 percentage points from baseline); prescribed by or in consultation with a hematologist; dosing is in accordance with the U.S. Food and Drug Administration (FDA) approved labeling; reauthorization will be for no more than 12 months.

Policy Summary

PayerUnitedHealthcare

PolicyRyplazim (Plasminogen, Human-Tvmh) (for Ohio Only)

Policy CodePolicy CSOH2025D0070.C

Change TypeNo material clinical or coverage changes

Effective DateMay 1, 2025

Next Review DateN/A

Key ActionObtain prior authorization with documentation of plasminogen activity ≤45%, clinical signs/symptoms, hematology prescriber or consultation, and dosing per FDA labeling.

SourceLink

On This Page

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Provider Action Items, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior Authorization Required. Prior authorization is required for Ryplazim (plasminogen, human-tvmh). Requests must include documentation of diagnosis, plasminogen activity level (≤ 45% for initial therapy), presence of clinical signs/symptoms, prescriber specialty, and dosing consistent with FDA labeling. Initial and reauthorization approvals will be for up to 12 months.

Affected HCPCS: J2998 (Injection, plasminogen, human-tvmh, 1 mg)
Required diagnosis code example: E88.02 (Plasminogen deficiency)

Step Therapy

No step therapy requirements specified. There are no mandated prior medications or trials required before coverage of Ryplazim.

Documentation Required

Required Clinical and Coding Documentation

Documentation required for prior authorization and medical review: include diagnosis of hypoplasminogenemia, laboratory plasminogen activity level with numeric result, clinical signs and symptoms (e.g., ligneous conjunctivitis, gingivitis, tonsillitis, abnormal wound healing), documentation of prior response to therapy for continuation requests (e.g., lesion size/number reduction, improved wound healing, plasminogen activity trough increase ≥ 10 percentage points from baseline), prescriber specialty (hematologist) or consultation note, and proposed dosing consistent with FDA labeling. Initial and reauthorization requests should specify duration requested (up to 12 months).

Denial Risk

Denial Triggers for Not Meeting Criteria

Denial triggers. Requests that do not meet all stated criteria may be denied. Common denial reasons include: plasminogen activity > 45% for initial therapy, absence of documented clinical signs or symptoms of hypoplasminogenemia, lack of hematology prescriber or consultation, dosing inconsistent with FDA-approved labeling, failure to document prior benefit for continuation requests, or requests for indications considered unproven (e.g., idiopathic pulmonary fibrosis).

Background and Clinical Context

Plasminogen is a liver-synthesized protein that is converted to plasmin, which mediates fibrin clot lysis. Plasminogen deficiency type 1 (hypoplasminogenemia) is a rare inherited disorder characterized by low plasminogen levels and formation of fibrin-rich ligneous pseudomembranous lesions on mucous membranes, commonly presenting as ligneous conjunctivitis and other mucosal lesions.

Replacement therapy with human plasminogen (Ryplazim) temporarily corrects the deficiency and can reduce extravascular fibrinous lesions; policy coverage is based on documented low plasminogen activity (≤ 45% of laboratory standard) and clinical signs or symptoms consistent with hypoplasminogenemia.

Definitions and Clinical Manifestations

inv-14: Hypoplasminogenemia — definition of plasminogen deficiency type 1 (rare autosomal-recessive disorder) and concise attributes

DefinitionPlasminogen deficiency type 1 (hypoplasminogenemia): a rare autosomal-recessive disorder of the fibrinolytic system causing low plasminogen levels and formation of fibrin‑rich ligneous pseudomembranous lesions on mucous membranes.

GeneticsInherited in an autosomal‑recessive manner

PathophysiologyLow plasminogen leads to abnormal extravascular accumulation or growth of fibrin‑rich lesions due to impaired fibrinolysis

Clinical significanceMost common manifestation is ligneous conjunctivitis which can progress to visual impairment or blindness if untreated

inv-15: Clinical manifestations — examples such as ligneous conjunctivitis, gingivitis, tonsillitis, abnormal wound healing

Ligneous conjunctivitisInflamed, woody growths on conjunctival membranes; most common manifestation and may lead to visual impairment or blindness

GingivitisFibrin‑rich pseudomembranous lesions affecting gingival mucosa

TonsillitisRecurrent or chronic fibrinous inflammation of tonsillar mucosa associated with plasminogen deficiency

Abnormal wound healingImpaired fibrinolysis resulting in persistent fibrinous lesions and delayed or abnormal healing

OpenPayer

Ryplazim (Plasminogen, Human-Tvmh) (for Ohio Only)

Coverage Criteria for Ryplazim (plasminogen, human-tvmh)

Coding and Diagnostic Codes

Provider Action Items, Prior Authorization, and Documentation

Background and Clinical Context

Definitions and Clinical Manifestations