- Precertification Criteria
Under some plans, including plans that use an open or closed formulary, Soliris is subject to precertification. If precertification requirements apply Aetna considers Soliris to be medically necessary for those members who meet ALL of the following precertification criteria:
- A documented diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), with flow cytometric confirmation of at least 10% PNH type III red cells or greater than 50% of glycosylphosphatidylinositol-anchored proteins (GPI-AP)-deficient polymorphonuclear cells (PMNs); AND
- Member has been vaccinated against meningococcal infection (at least 2 weeks prior to eculizumab treatment, if not previously vaccinated); AND
- Member does not meet diagnostic criteria for severe aplastic anemia (see special notes); AND
- Member meets one of the following criteria:
- Member is transfusion-dependent (i.e., has at least 1 transfusion in the 24 months prior to initiation of eculizumab due to documented hemoglobin less than 7 g/dL in persons without anemic symptoms or less than 9 g/dL in persons with symptoms from anemia) and has platelet counts of at least 30,000/microliter prior to initiation of eculizumab treatment; OR
- Member has a documented history of major adverse vascular events from thromboembolism (see special notes).
- A documented diagnosis of the treatment of atypical hemolytic uremic syndrome without serious unresolved Neisseria meningitis infection; AND
- The member has been vaccinated against meningococcal infection (at least 2 weeks prior to eculizumab treatment, if not previously vaccinated).
Paroxysmal nocturnal Hemoglobinuria (PNH)
A reduction in intravascular hemolysis as measured by:
- Stabilized hemoglobin levels
- Transfusion avoidance
Atypical hemolytic uremic syndrome (aHUS)
Reduction in signs of complement-mediated Thrombotic microangiopathy (TMA) including at least 2 of the following:
- Platelet count change from baseline
- Hematologic normalization (maintenance of normal platelet counts and LDH levels for at least 4 weeks)
- Complete TMA response (hematologic normalization plus at least 25% reduction in serum creatinine for at least 4 weeks)
- TMA-even free status (absence for at least 12 weeks of a decrease in platelet count of greater than 25% from baseline, plasma exchange or plasma infusion, and new dialysis requirement)
- Daily TMA intervention rate (defined as the number of plasma exchange or plasma infusion interventions and the number of new dialysis required per patient per day)
Note: While receiving eculizumab (Soliris), the member should be revaccinated according to current medical guidelines for vaccine use while on eculizumab (Soliris) therapy [REMS program]
Criteria for Diagnosis of Severe Aplastic Anemia:
The diagnostic criteria for severe aplastic anemia are:
- A bone marrow biopsy showing less than 25 percent of normal cellularity; or a bone marrow biopsy showing less than 50 percent normal cellularity in which fewer than 30 percent of the cells are hematopoietic; and
- At least two of the following are present: 1) absolute reticulocyte count less than 40,000/microliter; 2) absolute neutrophil count (ANC) less than 500/microliter; or 3) platelet count less than 20,000/microliter.
Source: Guinan, 2011.
Major Adverse Vascular Events (MAVE):
- Acute peripheral vascular occlusion,
- Clinically apparent distal embolization (e.g., lower extremity ulceration, tissue necrosis, gangrene, limb amputation or other end-organ damage)
- Deep vein thrombosis,
- Hepatic/portal vein thrombosis,
- Mesenteric/splenic vein thrombosis,
- Pulmonary embolus,
- Renal vein thrombosis,
- Cerebrovascular accident,
- Myocardial infarction,
- Transient ischemic attack,
- Unstable angina.
On September 23, 2011, the FDA approved eculizumab to treat patients with aHUS. The safety and effectiveness of eculizumab for the treatment of aHUS were established in two single-arm trials in 37 adults and adolescent patients with aHUS and one retrospective study in 19 pediatric patients and 11 adult patients with aHUS. Patients treated with eculizumab in these studies experienced a favorable improvement in renal function, including elimination of the requirement for dialysis in several patients with aHUS that did not respond to plasma therapy. Patients treated with eculizumab also exhibited improvement in platelet counts and other blood parameters that correlate with aHUS disease activity. The most common side effects observed in patients treated with eculizumab for aHUS included anemia, diarrhea, headache, hypertension, leukopenia, nausea, vomiting, as well as upper respiratory and urinary tract infections. This new indication for eculizumab is being approved with an extension of the existing Risk Evaluation and Mitigation Strategy (REMS), to inform health care professionals and patients about the known risk of life-threatening meningococcal infections. Eculizumab is contraindicated in patients with unresolved serious Neisseria meningitidis infection
Place of Service:
The above policy is based on the following references:
1. AHFS Drug Information® with AHFSfirstReleases®. ( www.statref.com), American Society Of Health-System Pharmacists®, Bethesda, MD. Updated periodically.
2. DRUGDEX® System [Internet database]. Greenwood Village, Colo: Thomson Micromedex. Updated periodically.
3. Drug Facts and Comparisons on-line. (www.drugfacts.com), Wolters Kluwer Health, St. Louis, MO. Updated periodically.
4. PDR® Electronic Library™ [Internet database]. Greenwood Village, Colo: Thomson Micromedex. Updated periodically.
5. Clinical Pharmacology [Internet database]. Gold Standard Inc. Tampa, FL. Updated periodically.
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.
March 21, 2017