The medications listed under this medical policy may be subject to the Redirection of Care Program. Some contracts may require medications to be provided in a non-hospital based setting. For more information, please visit https://www.bcbswy.com/providers/site-of-care/.
This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical circumstances may warrant individual consideration, based on review of applicable medical records.
I. Length of Authorization 13
- Initial: Prior authorization validity will be provided initially for 12 months (365 days), unless otherwise specified.
- Renewal: Prior authorization validity may be renewed every 12 months (365 days) thereafter, unless otherwise specified.
II. Dosing Limits
Max Units (per dose and over time) [HCPCS Unit]:
Emphysema due to alpha-1-antitrypsin (AAT) deficiency
• 2800 billable units every 28 days aGVHD
• 700 billable units for a total of 8 doses in 28 days (5600 billable units per 28 days)
III. Initial Approval Criteria 1-5
Prior authorization validity is provided in the following conditions:
- Member is at least 18 years of age; AND
Universal Criteria 1-5
- Member does NOT have any FDA labeled contraindications to the requested agent; AND
Emphysema due to alpha-1-antitrypsin (AAT) deficiency † (Ф – orphan designation applies only to Prolastin-C) 1-6,7,8,10,14-17
- Member has an FEV1 in the range of 30-65% of predicted; AND
- Member has alpha-1-antitrypsin (AAT) deficiency with PiZZ, PiZ (null), or Pi (null, null) phenotypes; AND
- Member has clinical evidence of panacinar/panlobular emphysema or centrilobular emphysema; AND
- Member has low serum concentration of AAT ≤ 57 mg/dL (measured by nephelometry) or ≤ 11 µM/L (measured by Enzyme-Linked Immunosorbent Assay [ELISA]); AND
- Member is not a tobacco smoker; AND
- Member is receiving optimal medical therapy (e.g., pharmacologic therapy [e.g., bronchodilators, inhaled corticosteroids, etc.], comprehensive case management, pulmonary rehabilitation, vaccinations, smoking cessation, self-management skills, supplemental oxygen, etc.)
† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug
IV. Renewal Criteria 1-5
Prior authorization validity may be renewed based upon the following criteria:
- Member continues to meet the universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
- Duration of authorization has not been exceeded (refer to section I); AND
- Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: severe hypersensitivity reactions, etc.; AND
Emphysema due to alpha-1-antitrypsin (AAT) deficiency 1-7,17
- Disease response with treatment as defined by elevation of AAT levels above baseline, substantial reduction in rate of deterioration of lung function as measured by percent predicted FEV1, or improvement in CT scan lung density
V. Dosage/Administration 1-5,13
Indication |
Dose |
Emphysema due to AAT deficiency |
Administer 60 mg/kg intravenously once weekly |
Acute GVHD |
Administer 60 mg/kg intravenously on days 1, 4, 8, 12, 16, 20, 24, and 28 for up to 4 consecutive weeks (maximum of 8 doses) |
J0256 - Aralast
J0257 - Glassia
J0256 - Prolastin-C
J0256 - Zemaira
ICD-10 |
ICD-10 Description |
D89.810 |
Acute graft-versus-host disease |
D89.812 |
Acute on chronic graft-versus-host disease |
D89.813 |
Graft-versus-host disease, unspecified |
E88.01 |
Alpha-1-antitrypsin deficiency |
T86.09 |
Other complications of bone marrow transplant |
Non-quantitative treatment limitations (NQTLs) refer to the methods, guidelines, standards of evidence, or other conditions that can restrict how long or to what extent benefits are provided under a health plan. These may include things like utilization review or prior authorization. The utilization management NQTL applies comparably, and not more stringently, to mental health/substance use disorder (MH/SUD) Medical Benefit Prescription Drugs and medical/surgical (M/S) Medical Benefit Prescription Drugs. The table below lists the factors that were considered in designing and applying prior authorization to this drug/drug group, and a summary of the conclusions that Prime’s assessment led to for each.
Factor |
Conclusion |
Indication |
Yes: Consider for PA |
Safety and efficacy |
No: PA not a priority |
Potential for misuse/abuse |
No: PA not a priority |
Cost of drug |
Yes: Consider for PA |
1. Glassia [package insert]. Cambridge, MA; Takeda Pharmaceuticals USA, Inc.; February 2025. Accessed March 2026.
2. Zemaira [package insert]. Kankakee, IL; CSL Behring LLC; January 2024. Accessed March 2026.
3. Aralast NP [package insert]. Cambridge, MA; Takeda Pharmaceuticals USA, Inc.; May 2025. Accessed March 2026.
4. Prolastin-C Liquid [package insert]. Research Triangle Park, NC; Grifols Therapeutics, LLC; May 2020. Accessed March 2026.
5. Prolastin-C [package insert]. Research Triangle Park, NC; Grifols Therapeutics, LLC; January 2022. Accessed March 2024.
6. American Thoracic Society/European Respiratory Society Statement: Standards for the Diagnosis and Management of Individuals with Alpha-1 Antitrypsin Deficiency. American Thoracic Society; European Respiratory Society. Am J Respir Crit Care Med. 2003 Oct 1;168(7):818-900.
7. Sandhaus RA, Turino G, Brantly ML, et al. The diagnosis and management of alpha-1 antitrypsin deficiency in the adult. Chronic Obstr Pulm Dis (Miami). 2016; 3(3):668-682.
8. Marciniuk DD, Hernandez P, Balter M, et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2012;19(2):109-16.
9. Stocks JM, Brantly M, Pollock D, et al. Multi-center study: the biochemical efficacy, safety and
tolerability of a new α1-proteinase inhibitor, Zemaira. COPD. 2006;3:17–23.
10. Miravitlles M, Dirksen A, Ferrarotti I, et al. European Respiratory Society statement: diagnosis
and treatment of pulmonary disease in α1-antitrypsin deficiency. Eur Respir J 2017; 50.
11. Referenced with permission from the NCCN Drugs & Biologics Compendium (NCCN Compendium®) for Alpha1-Proteinase Inhibitor (Human). National Comprehensive Cancer Network, 2026. The NCCN Compendium® is a derivative work of the NCCN Guidelines®. NATIONAL COMPREHENSIVE CANCER NETWORK®, NCCN®, and NCCN GUIDELINES®
are trademarks owned by the National Comprehensive Cancer Network, Inc.” To view the most recent and complete version of the Compendium, go online to NCCN.org. Accessed March 2026.
12. Referenced with permission from the NCCN Drugs & Biologics Compendium (NCCN Compendium®) Hematopoietic Cell Transplantation (HCT). Version 3.2025. National Comprehensive Cancer Network, 2025. The NCCN Compendium® is a derivative work of the NCCN Guidelines®. NATIONAL COMPREHENSIVE CANCER NETWORK®, NCCN®, and
NCCN GUIDELINES® are trademarks owned by the National Comprehensive Cancer Network, Inc.” To view the most recent and complete version of the Compendium, go online to NCCN.org. Accessed March 2026.
13. Magenau JM, Goldstein SC, Peltier D, et al. α1-Antitrypsin infusion for treatment of steroid-resistant acute graft-versus-host disease. Blood. 2018 Mar 22;131(12):1372-1379. doi: 10.1182/blood-2017-11-815746.
14. Global Initiative for Chronic Obstructive Lung Disease (GOLD). Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. Global Initiative for Chronic Obstructive Lung Disease (GOLD); 2026 Report.
15. Bornhorst JA, Greene DN, Ashwood ER, et al. α1-Antitrypsin phenotypes and associated serum protein concentrations in a large clinical population. Chest. 2013 Apr;143(4):1000-1008. doi: 10.1378/chest.12-0564. PMID: 23632999.
16. Feitosa PHR, Castellano MVCO, Costa CHD, et al. Recommendations for the diagnosis and treatment of alpha-1 antitrypsin deficiency. J Bras Pneumol. 2024 Dec 6;50(5):e20240235. doi: 10.36416/1806-3756/e20240235. PMID: 39661838; PMCID: PMC11601085.
17. Hernandez P, Bosse Y, Bush P, et al. Alpha-1-Antitrypsin Deficiency Targeted Testing and Augmentation Therapy: A Canadian Thoracic Society Meta-Analysis and Clinical Practice Guideline. April 2025. CHEST, Volume 167, Issue 4, P1044-1063.
Drug |
Manufacturer |
HCPCS code |
1 Billable Unit |
SDV Size |
NDC |
Aralast NP (powder) |
Takeda Pharmaceuticals USA Inc. |
J0256 |
10 mg |
1 g/50 mL |
00944-2815-xx |
0.5 g/25 mL |
00944-2814-xx |
||||
Glassia (solution) |
Takeda Pharmaceuticals USA Inc. |
J0257 |
10 mg |
1 g/50 mL |
00944-2884-xx |
Prolastin-C Liquid (solution) |
Grifols Therapeutics LLC |
J0256 |
10 mg |
500 mg/10 mL |
13533-0705-xx |
1 g/20 mL |
|||||
4 g/80 mL |
|||||
Zemaira (powder) |
CSL Behring LLC |
J0256 |
10 mg |
1 g/20 mL |
00053-7201-xx |
4 g/76 mL |
00053-7202-xx |
||||
5 g/95 mL |
00053-7203-xx |
